>0« 






"of 















^ ••••• ,# ©*. •Sri-* ^ 



* d* 








* ^ 



P *»V* 








4> O * 




& 



- - 







"oV* 








/% 




*^ -^ 













** %■ 




<?\ 



++# 





•% v T 







++$ 









M 





4 O 




"of 




l<2» 




«bV* 

























A TREATISE 



ON THE 



Nervous Diseases of Children 



PHYSICIANS AND STUDENTS 



BY 

B. SACHS, M.D. 



PROFESSOR OF MENTAL AND NERVOUS DISEASES IN THE NEW YORK POLYCLINIC ; CONSULTING 

NEUROLOGIST TO THE MT. SINAI HOSPITAL; NEUROLOGIST TO THE MONTEFIORE 

HOME FOR CHRONIC INVALIDS; EX-PRESIDENT OF THE 

AMERICAN NEUROLOGICAL ASSOCIATION 




NEW YORK 
WILLIAM WOOD AND COMPANY 

1895 



21-ttfZ^ 



i 



J" 



COPYKIGHT, 1895, BY 

WILLIAM WOOD AND COMPANY 



TROW DIRECTORY 

PRINTING AND BOOKBINDING COMPANY 

NEW YORK 



PREFACE. 

A little more than two years ago a fellow-neurologist 
suggested to the author that it would be well for him to 
write a treatise on the Nervous Affections of Childhood, 
which would give both the physician and the student fuller 
information regarding these diseases than is to be obtained 
from text-books on pediatrics. 

The first conception of what such a treatise ought to be 
was a very modest one, but the task has grown upon the 
author's hands until the book assumed its present propor- 
tions. It was thought best to include all those diseases 
which either occur frequently during early life, or which, 
when occurring at this period, have some distinctive feat- 
ures. According to this plan such affections as epilepsy, 
tumors of the brain, and meningitis, which occur both in 
adult life and in childhood, have been treated fully, but tabes 
dorsalis and general paresis, although observed occasion- 
ally in youthful individuals, did not seem to come within 
the scope of this treatise. 

In arranging the chapters the effort has been made to 
indicate by their sequence the natural relation of the vari- 
ous diseases. No apology is needed for the Introductory 
Chapter, which the author, from his experience as a teacher, 
knows will meet the needs of the practitioner and the stu- 
dent. Contrary to the usual custom, the functional disor- 
ders of the nervous system are discussed first. There 
seems to be good reason for this. These functional dis- 
orders are of the greatest practical importance and consti- 
tute fully one-half of the nervous diseases observed during 
early years. Moreover, several of these disorders, such as 
convulsions, epilepsy, and hysteria, have an important bear- 
ing upon the entire life of the child, and are so closely re- 



IV PREFA CE. 

lated to many organic diseases of the nervous system that 
it seemed absolutely necessary to explain these functional 
troubles before proceeding to the discussion of structural 
diseases. A number of less frequent functional conditions 
were necessarily included in the first division of the book 
in order to preserve the proper continuity of subjects. 

Organic diseases of the peripheral nerves, of the spinal 
cord, and of the brain, have been placed under one large 
subdivision. The plan of beginning with the peripheral 
nerve troubles, and then proceeding from the simpler to 
the more complex, has been adopted by many writers in 
this country and abroad. 

The reader is referred to larger text-books on nervous 
diseases for detailed descriptions, yet the author has deemed 
it expedient to give a short, but sufficient, account of the 
anatomy, physiology, and pathology of the chief divisions 
of the nervous system. The chapters upon the mental dis- 
orders of childhood have been introduced partly for the 
sake of completeness, partly because the psychic disturb- 
ances of early life are frequently overlooked or but poorly 
understood. 

While especial attention has been given to the pathology 
and diagnosis of the diseases under discussion, the impor- 
tance of giving full details of treatment has been kept 
steadily in mind. The author has in almost every instance 
preferred to give the treatment which his own experience 
approves of rather than to burden the book Avith a list of 
therapeutic measures which have been tried and found to 
be of questionable value. The Appendix will explain itself. 

The short bibliographies have the twofold purpose of 
indicating the sources from which the author has drawn 
some of his facts, and of acquainting the physician with the 
most important writings on any subject which he may 
wish to study in detail. On this account it was natural to 
consider recent literature more fully than the older, but 
wherever it was possible all the chief authorities have been 
quoted. 

The author believes that almost every chapter bears 
the impress of a large experience, and reveals opinions 
that result from his own- researches and studies as modified 



2i East Sixty-fifth Street, New York. 



* To express these obligations fully, he would have to refer to the labors of Jack- 
son, Ross, Gowers, Bramwell, Buzzard, and many others in Great Britain ; to those 
of Duchenne, Charcot, Grasset, Marie, Brissaud, Bourneville, and Dejerine in France ; 
to the writings of Erb, Westphal, Strumpell, Eulenburg, Oppenheimer, Freud, Hirt, 
Moebius, and others in Germany ; and to those of Mitchell, Osier, Dana, Starr, 
Mills, Gray, Knapp, Seguin, Collins, Herter, and Peterson in America. 



PRE FA CE. V 

by a conscientious reading of neurological literature. He 
feels a deep sense of obligation to the many excellent in- 
vestigators who have contributed so much to the elucida- 
tion of nervous and mental disorders.* It is particularly 
gratifying to be able to acknowledge the amount of earn- 
est work which has been done in this special department 
of neurology by American physicians. 

Especial thanks are due to two neurologists of this city 
who have given invaluable assistance. Dr. Joseph Collins 
has undertaken the arduous task of reading manuscript 
and revising proof. He has favored the author with much 
valuable criticism and many excellent suggestions regard- 
ing the proper presentation of facts, and has also been good 
enough to prepare an ample index. Dr. Alfred Wiener has 
devoted a great deal of time and labor in the most gener- 
ous manner to the preparation of photographs and micro- 
scopical specimens, and has assisted very largely in the 
revision and collection of clinical histories. The writer is 
also deeply indebted to his publishers for the interest taken 
in the preparation of this book and for the liberality dis- 
played in furnishing illustrations. The sources of these 
illustrations have been carefully stated ; where no such 
reference is given, the illustrations are the author's own. 



CONTENTS. 



CHAPTER I. 

PAGE 

Introduction — Methods of Examination, i 

Examination schemes, 3 ; cranial measurements, 5 ; Preyer's observations, 
7; visual tests, 8 ; action of muscles, 11-34; gait, 35 ; sensory distribu- 
tion, 36 ; reflexes, 40 ; electrical examination, 43. 



PART I. 

GENERAL NERVOUS DISEASES. 

CHAPTER II. 

Convulsions — Eclampsia Infantum, 49 

Causes, 50; theories, 53; symptoms, 56; Laryngismus stridulus, 57; diag- 
nosis of convulsions, 60 ; treatment, 62. 

CHAPTER III. 
Epilepsy, . 65 

Symptoms of "grand mal," 66; "petit mal," 68; causes, 69; diagnosis, 
73 ; prognosis, 74 ; pathological anatomy, 77 ; treatment, 78 ; surgical 
treatment, 81. 

CHAPTER IV. 
Hysteria, S5 

Psychic or mental hysteria, 86; motor manifestations, SS ; distinction be- 
tween epilepsy and hystero-epilepsy, 91 ; hysterical paralysis, 93; apho- 
nia, 94 ; sensory symptoms, 97 ; visceral hysteria, 100 ; diagnosis of 
hysteria, 102 ; pathology, 103 ; duration and courses, 103 ; treatment, 
104. 



Vlii CONTENTS. 

CHAPTER V. 

Chorea, 109 

Etiology, 109; causes, 111 ; symptoms, 113 ; laryngeal chorea, 115 ; com- 
plications in chorea, 117; duration, 118; diagnosis, 119; morbid anat- 
omy, 120; prognosis, 124; treatment, 125. 

CHAPTER VI. 

Choreiform Diseases, 131 

Hereditary or Huntington's chorea, 131; symptoms, 132; pathological 
anatomy, 134; treatment, 135; Hereditary chorea without dementia, 
135; Habit chorea, 136; complex Co-ordinated movements (complex 
tics), 139; Gyrospasms of the head, 137; Chorea electrica, 138; Mala- 
die des tics convulsifs, 139; treatment, 140; Thomsen's disease, 141 ; 
Congenital Paramyotonia, 143 ; Paramyoclonus multiplex, 143. 

CHAPTER VII. 

Tetanus, 147 

Etiology, 147; symptoms, 149; tetanus neonatorum, 151; pathological 
anatomy and morbid pathology of tetanus, 153 ; differential diagnosis, 
156; prognosis, treatment, 157. 

CHAPTER VIII. 
Tetany, 160 

Symptoms, 161; etiology, 163; symptoms of latent period, 164; differen- 
tial diagnosis, 165 ; morbid anatomy and pathology, 165 ; treatment, 
167; tetanoid chorea, 168. 

CHAPTER IX. 
Headaches, 170 

Classification, 170; anaemic headaches, 171 ; neurasthenic headaches, 
173; headaches due to transitory hyperemia, 174; gastric headaches, 
175; headaches due to genital irritation, 175; to ear disease, 175; 
to organic disease of the brain, 176; in acute infectious diseases, 177; 
malarial headaches, 177; uraemic headaches, 178; toxic headaches, 178; 
those due to eye-strain, 178; Migraine, 179; etiology, 182; pathology, 
183; relation to eye-strain, 185; diagnosis, 186; prognosis, 186; 
treatment, 187. 

CHAPTER X. 
The Disorders of Sleep, . . . 191 

Normal sleep, 191; causes of insomnia, 192; Pavor nocturnus, 193; Enu- 
resis nocturna, 195 ; Somnambulism, 196. 



CONTENTS. IX 



CHAPTER XI. 

PAGE 

Vasomotor and Tropho-neuroses, 197 

Exophthalmic goitre, 197 ; symptoms, 198 ; morbid anatomy, 202 ; treat- 
ment, 203 ; Thyroidectomy, 204 ; Thyroid enlargement at the age of 
puberty, 305 ; Tachycardia, 205 ; Myxoedema, 205 ; Angio-neurotic 
oedema, 207; Raynaud's disease, 209; Facial hermiatrophy, 210. 



PART II. 

ORGANIC DISEASES OF THE NERVOUS SYSTEM. 

CHAPTER XII. 

Diseases of the Peripheral Nerves, . . . . . . .217 

Some peripheral palsies, 217; anatomy and pathology of peripheral 
nerves, 217 ; brachial plexus lesions, 221 ; Erb's and Klumpke's types, 
222; Obstetrical palsies, 223 ; pathology, 224; diagnosis, 225 ; progno- 
sis, 226 ; treatment, 227 ; Paralysis of the lower limbs, 228 ; Facial pal- 
sy, 229 ; treatment, 236 ; other peripheral palsies, 238 ; Spasm, Tic 
convulsif, Wry neck, 238. 

CHAPTER XIII. 
Multiple Neuritis, 240 

Symptoms, 240; course, 245; toxic forms, 246; toxsemic forms, 247; 
malarial neuritis, 247 ; pathological anatomy of multiple neuritis, 24S ; 
diagnosis, 249 ; treatment, 251 ; Diphtheritic paralysis, 253 ; pathologi- 
cal anatomy, 256; diagnosis, 257; treatment, 258; Lead paralysis, 259; 
diagnosis and treatment, 261. 

DISEASES OF THE SPINAL CORD. 

CHAPTER XIV. 

Anatomy, Physiology, and Pathology of the Spinal Cord, . . 263 

Structure of the cord, 263 ; of gray matter, 2C5 ; of white matter. 268 ; 
pyramidal tracts, 270; division of white columns, 271 ; course of ante- 
rior and posterior root-fibres, 274; localization in the spinal cord, 277 ; 
functions of spinal cord, 279; blood-supply of spinal cord, 281, 



CONTENTS. 



CHAPTER XV. 

PAGE 

An/Emia and Hyperemia of the Spinal Cord, 284 

Diagnosis, 284 ; degeneration of cord in pernicious anaemia, 285 ; hyper- 
emia, 286. 



CHAPTER XVI. 

Infantile Spinal Paralysis — The Essential Paralysis of Chil- 
dren — Poliomyelitis Anterior Acuta, 289 

Symptoms, 289 ; distribution of paralysis, 292 ; deformities, 296 ; mor- 
bid anatomy and pathology, 298 ; theory of the disease, 303 ; differen- 
tial diagnosis, 304 ; prognosis, 307 ; treatment, 308 ; Subacute anterior 
poliomyelitis, 310. 



CHAPTER XVII. 

Acute Myelitis 313 

Symptoms, 313 ; origin of myelitis, 318 ; pathology and morbid anatomy, 
319; differential diagnosis, 322 ; prognosis, 324; treatment, 325 ; Trau- 
matic injuries of the spinal cord, 328 pathology, 330 ; treatment, 331 ; 
Landry's paralysis, 332. 

CHAPTER XVIII. 

Syphilis of the Spinal Cord : Specific Myelitis and Meningo-mye- 
litis, 333 

Symptoms, 333 ; Erb's type, 334 ; hereditary syphilitic affections, 336 ; dif- 
ferential diagnosis, 337 ; morbid anatomy, 338 ; prognosis, 341 ; treat- 
ment, 342. 

CHAPTER XIX. 
Disseminated Sclerosis, 345 

Symptoms, 345 ; pathological anatomy, 349 ; atypical forms and differen- 
tial diagnosis, 351 ; paralysis agitans and multiple sclerosis, 353; prog- 
nosis and treatment, 354. 



CHAPTER XX. 

Compression of the Spinal Cord — Pott's Paralysis, . . . 357 

Causes of injury to the cord in Pott's disease, 358; symptoms of Pott's 
paralysis, 359; diagnosis, 360; prognosis, treatment, 361. 



CONTENTS. xi 



CHAPTER XXI. 

PAGE 

Tumors of the Spinal Cord and its Meninges, 364 

Causes and symptoms, 364 ; pathology, diagnosis, 369 ; prognosis, treat- 
ment, 371 ; Syringomyelia and gliosis of the cord, 374. 



CHAPTER XXII. 

Hereditary or Family Diseases of the Spinal Cord, . . .377 

Hereditary ataxy. — Friedreich's disease, 378 ; symptoms, 379 ; differential 
diagnosis, 385 ; pathological anatomy, 386 ; Hereditary ataxy (cerebellar 
type; type Nonne-Marie), 390; Hereditary spastic paralysis, 391; spi- 
nal type, 392 ; diagnosis, 395 ; cerebral type, 396 ; cerebral diplegia, 
39S ; morbid anatomy of the cerebral type of hereditary spastic paral- 
ysis, 398 ; classification of hereditary spinal diseases, 401. 

CHAPTER XXIII. 
Progressive Muscular Atrophies, 404 

Division of the subject, 405 ; distinction between progressive amyotro- 
phies and progressive myopathies, 406 ; type Aran-Duchenne, 407 ; He- 
reditary progressive muscular atrophy (Hoffmann), 409 ; Progressive 
neural muscular atrophy, etc., 411 ; etiology, diagnosis, 416 ; pathol- 
ogy, 418; Primary myopathies, 420; types of primary dystrophies, 
421; Muscular pseudo-hypertrophy, 423; Erb's type, or the juvenile 
form, 426 ; Landouzy-Dejerine type, 426 ; diagnosis of dystrophies, 
429; physiological hypertrophy, 429; pathology, 431; histological 
changes in muscles, 431 ; duration, treatment, 435 ; Total absence and 
early atrophy of muscles, 436. 



CHAPTER XXIV. 

Malformations and Conditions due to Defective Development of 
the Cord, 439 

Amyelia, Atelomyelia, Asymmetry, Heterotopia, 439 ; Diplomyelia, 440 ; Spina 
bifida, 440; symptoms, 442; treatment, 443. 



DISEASES OF THE BRAIN. 

CHAPTER XXV. 
Anatomy, Physiology, and Pathology of the Brain, . . . 445 

Structure and function of cortex, 445 ; relation of brain areas to skull. 
44S ; Reid's lines, 450; the motor tract, 450; symptoms o\ cortical and 



Infantile Cerebral Palsies (Spastic Hemiplegia, Diplegia, Para- 
plegia), 

History of subject, 523; frequency, 524; distribution of paralysis, 526; 
onset, 527; etiology, 52S; form of palsy, 531; rigidities and contrac- 
tures, 533; gait, 534; post-paralytic disturbances of motion, 534; atro- 
phy of muscles, 535 ; epilepsy with cerebral palsy, 536 ; idiocy with 
same, 537 ; classification of palsies, 538 ; morbid anatomy, 549 ; of 
congenital cases, 539; of birth palsies, 540; of acute cerebral palsies, 
542 ; polio-encephalitis, 547 ; differential diagnosis, 548 ; prognosis, 
549; treatment, 550; surgical treatment, 553. 



PAGE 



Xll CONTENTS. 

internal capsule lesions, 455 ; sensory tract, 45S ; visual tract, 459 ; ol- 
factory and auditory tracts, 461 ; arrangement of cranial nerve nuclei, 
462 ; degeneration, 463 ; the blood-supply of the brain, 464 ; venous 
circulation, 463 ; weight of brain, 471 ; speech defects — aphasia, 471; 
defective speech development, 472 ; aphasia, motor and sensor}-, 473 ; 
further subdivisions, 477 ; various theories, 47S ; occurrence of aphasia, 
4S0; tests for aphasia, 4S1. 



CHAPTER XXVI. 

Meningitis and Encephalitis, . 4S3 

Simple acute meningitis, 4S3 ; etiology, morbid anatomy, 4S5 ; diagnosis, 
4S6 ; prognosis, treatment, 4S7; Tubercular meningitis, 4S9 ; onset, 
4S9 ; symptoms, 490 ; morbid anatomy, 492 ; pathology, diagnosis, 
493; prognosis, 494; treatment, 495; Epidemic cerebro-spinal menin- 
gitis, 496; microbic origin, 496; history of epidemics, 497; symptoms, 
499 ; morbid anatomy, etc. , 501 ; differential diagnosis, treatment, 502 ; 
Meningitis due to traumatism after operation, 503 ; Meningitis due to 
ear disease, 504 ; Meningitis after infectious diseases, 506 ; after in- 
fluenza, 507 ; Septicemic meningitis, 507 ; Acute encephalitis, 503 ; 
Polio-encephalitis superior, 509; Bulbar palsies, 510. 



CHAPTER XXVII. 

Hydrocephalus, 514 

Acute hydrocephalus, 514; meningitis serosa, 514; pathology of acute 
hydrocephalus, 515; chronic hydrocephalus, 516; congenital hydro- 
cephalus, 517; acquired internal hydrocephalus, 519; prognosis, treat- 
ment, 521 ; surgical methods, 522. 



CHAPTER XXVIII. 



523 



CONTENTS. Xlll 



CHAPTER XXIX. 

PAGE 

Tumors of the Brain and its Meninges, 556 

Forms of tumor, locations of tumors, 556; etiology, 557; symptoms, 
558; Macewen's symptom, 560; tumors of cortex, 560; of frontal 
lobe, of third frontal convolution, of motor area, 561 ; of parietal lobe, 
of occipital lobe, of temporo-sphenoidal lobe, 562 ; of basal ganglia, of 
cms cerebri, of the corpora quadrigemina, 563; of the pons, 565 ; of 
the cerebellum, 566; differential diagnosis, 568; pathology, 569; 
treatment, 572; surgical procedures, 574. 



CHAPTER XXX. 
Abscess of the Brain, 576 

Occurrence of, 576 ; connection with ear disease, 578 ; with nasal disease, 
578; symptoms of abscess, 579; differential diagnosis, 581; prognosis, 
582; treatment, 583; surgical procedures, 584; Thrombosis of the intra- 
cranial sinuses, 585 ; symptoms, 586; special symptoms of thrombosis, 
of cavernous, petrosal, and lateral sinuses, 587; treatment, 587. 



CHAPTER XXXI. 

Diseases and Conditions due to Defective Development of the 
Brain, 5S9 

Larger defects, 589; Cyclops, 589; Anencephalus, Hemicephalus, 590; 
Porencephaly, 590; congenital porencephaly, 591; acquired poren- 
cephaly, 593 ; Microcephalus, 594 ; craniectomy, Lannelongue's pro- 
cedure, 597 ; Partial cerebral defects, 599 ; defective development of the 
occipital lobe, 599 ; Agenesis corticalis, 601 ; Macrocephalus, 602 ; De- 
fective development of cranial nerve nuclei, 603 ; Pleuroplegia, 605. 



CHAPTER XXXII. 
Insanity, . 60S 

Differences between insanity of child and of adult, 60S ; frequency. 609 , 
etiology, 609; forms of insanity, 611 ; Imperative concepts, 611 ; Mi- 
sophobia, 612; Delire du toucher, 612; Agoraphobia, Cerebral neu- 
rasthenia, 613 ; Hypochondriasis, 615 ; Mania, 616; Melancholia, 618 ; 
Periodic and circular insanity, 621 ; Cataleptic insanity, 622 ; Acute de- 
mentia, 623; Paranoia, 623; Moral insanity, 625; Epileptic insanity. 
626; Paretic dementia, 627; Masturbation and insanity. 628 ; prog- 
nosis, 62S ; treatment. 629. 



XIV CONTENTS. 



CHAPTER XXXIII. pAGE 

Idiocy and Imbecility, 631 

Classification, 632 ; hereditary congenital idiocy, 632 ; developmental, 
acquired idiocy, 633 ; causes, 633 ; symptoms, 636 ; pathology, 641 ; 
prognosis, treatment, 643 ; Myxcedematous idiocy — sporadic cretinism, 
644 ; diagnosis, prognosis, treatment, 646. 



APPENDIX. 

A Few Therapeutic Suggestions, . . . . . . . 651 

The rest cure, 651 ; Hydrotherapy, 653 ; Drugs commonly used in nervous 
disorders, 655. 

INDEX, . 657 



LIST OF ILLUSTRATIONS. 



3- 
4- 

5- 

6. 

7- 
8. 

9. 
n. 

12. 

13. 

14. 
15. 
16. 

17. 
18. 

19. 



27. 
28. 
29. 
30- 



FIGURE 

i. Craniometrical Lines, 

2. Asymmetry of Skull, . _ 

Normal Visual Fields for Colors, 

Normal Visual Field, 

Field of Vision in Hemianopsia, 

Sensory Chart of Face, . 

Motor Points of Face, 

Patient with Hypertrophy of Infra and Supra Spinati, etc., 

10. Patient with Progressive Muscular Dystrophy, . 

Boy with Multiple Neuritis, . . . . 

Appearance of Hand in Early Stage of Progressive Muscular 

phy> 

Motor Points on Shoulder and Arm, 

Motor Points on Forearm and Hand, 

Motor Points on Inner Surface of Arm, . 

" Ape Hand," 

Extreme " Main en Griffe," 

Slight Atrophy of Interossei, . 

20. Motor Points on Thighs and Legs, . 
21 to 25. Deformities of Feet, 
26. Pes Equinus, ...... 

Pes Equinus of Eight Years' Duration, . 

Motor Points on Anterior Surface of Leg 

Motor Points on Trunk, .... 

Patient showing Lordosis, 



Atro 



of the 



in 



Deeeneration of the 



Small 



31 to 37. Distribution of Sensory Nerves, 

38. Child in Convulsive Seizure, 

39. Diffuse Neuroglia Sclerosis in Epilepsy 

40. Various Phases of the Earlier Stages 

Ganglion Cells, 

41. A Group of More Mature Neuroglia Cells 

Pyramids, 

42. Epileptoid Stage of Hystero-epileptic Attack. 

43. The Three Types of Distribution of Anaesthesia in Hysteria 

44. Hysterical Loss of Color Sense and Limitation of Visual Field 

45. Dilatation of Blood-vessels in White Matter of the Convolutions in 

Chorea, 



the Layer of 



S. 



PAGE 

5 

6 



9 
14 
17 
17 
19 
21 

21 
23 
23 
25 
25 
27 
27 
29 
3i 
32 
32 
32 
33 
34 
39 
50 
76 



76 

77 
90 

99 
100 



XVI 



LIST OF ILLUSTRATIONS. 



46. Changes in Purkinje's Cells in Chorea, . 

47. Tetanus Bacilli and Spores, 

48. Position of Hands in the Spasm of Tetany, 

49. Location of Head Pains, .... 

50. Case of Myxcedema with Idiocy, 
51 to 55. Normal and Degenerated Nerve Fibres, 

56. The Brachial Plexus ; Branches involved in Upper-Arm Type, 

57, 58. Anaesthetic Areas of Hands after Section of Ulnar Nerve, 

59. Palmar Surface after Section of Median Nerve, 

60. Relation of Seventh, Eighth, and Glosso-pharyngeal Nerves, etc. 

61. Boy with Facial Palsy in Stage of Recovery, . 

62. Young Boy with Multiple Neuritis, 

63. Chronic Interstitial Neuritis, 

64. Diagram showing the Relation of the Vertebral Spines, etc., 

65. Outlines of IV. and V. Cervical Segments, 

66. Cross Section of Sixth Cervical Segment of a Boy, Two Years Old, 

67. Diagram showing the Probable Relation of Cells and Fibres, etc. , . 

68. Descending Degeneration after a Lesion in the Internal Capsule, . 
Plate I. Fig. I. Sensory Tract, 

Fig. II. Cross Section of Cord, 

Fig. III. Relation of Motor Tract to Nuclei of Cranial 
Nerves, 

69. Secondary Degeneration Following a Lesion in the Left Cerebral 

Hemisphere, 

70. Diagrammatic Cross Section of Spinal Cord, 

71. Course of Sensory Fibres from the Posterior Roots to the Medulla 

Oblongata, 

72. Division of Sensory Root Fibres — Human Embryo of Six Months, . 

73. Blood Supply of Spinal Cord, 

74. Case of Infantile Spinal Palsy, 

75. Case of Acute Infantile Cerebral Palsy, 

76. Paralysis of Upper Arm with Atrophy, etc., 

Section through Lumbar Segment in Acute Poliomyelitis, 

Poliomyelitis Anterior of Old Standing, etc., 

Poliomyelitis Anterior ; Chronic Stage, etc., 

Secondary Ascending and Descending Degeneration, 

Sketch of Section of Spine in a Case of Fracture Dislocation of the 

Seventh Cervical Vertebra, 

82. Section through Pons showing Gummatous Infiltration with con- 

siderable Destruction of Tissue, 

83, 84. Sections showing Specific Lepto-meningitis, . . . 339, 

85. Degeneration of Cauda Equina in Multiple Sclerosis, 

86. Sclerotic Patches in Multiple Sclerosis, 

87. Vicinity of Locus Cceruleus in Multiple Sclerosis, .... 

88. Patient with Paralysis Agitans, who also exhibited some Symptoms 

of Disseminated Sclerosis 



PAGE 

123 

154 
161 
171 
206 
218 
219 
221 
222 
229 
231 
241 
249 
264 
265 
266 
267 
269 
270 
270 

270 

270 
273 

275 
276 
281 
290 
291 

293 
300 
301 
302 
314 

329 

333 
340 
348 
349 
35o 

353 



LIST OF ILLUSTRATIONS. XV 11 

FIGURE PAGE 

89. Section through Seventh Cervical Segment, showing Gliosis of 

Cord, 374 

90. Section through Part of Fourth Dorsal Segment in Gliosis of Cord, 375 

91. Case of Friedreich's Disease, ........ 378 

92. Same Case, showing Marked Atrophy of Shoulder Muscles, . . 379 

93. Deformity of Feet in Friedreich's Disease, ..... 382 

94. Section through Middle Dorsal Region of a Case of Friedreich's 

Disease, 387 

95. Section through Cervical Segment in same Disease, . . . 388 

96. Brain in Cerebral Type of Hereditary Spastic Paralysis, . . . 397 

97. Section through Cervical and Dorsal Segments in a Case of Me- 

ningoencephalitis. Probable defective development of pyram- 
idal tracts, etc., . . . . . . . . . 399 

98. Section through Motor Cortex in a Case of Spastic Paraplegia, . 400 

99. Diagrammatic Representations of Divisions of Progressive Mus- 

cular Atrophy, .......... 405 

100. Two Brothers Afflicted with Peroneal Form of Progressive Muscu- 

lar Atrophy, 412 

101, 102. Case of Peroneal Form of Progressive Muscular Atrophy, 414, 415 
103 to 105. Case of Progressive Muscular Atrophy in a Child with a 

Spinal Lesion, 417 

106 to 108. Three Brothers, illustrating Various Types of Progressive 

Muscular Dystrophies, 422-424 

109. Boy with Pseudo-Hypertrophy attempting to Straighten Himself, . 425 

1 10. Patient with Landouzy-Dejerine Type, 426 

in, 112. Changes in Muscular Tissue in a Case of Primary Dystrophy, 433 
113, 114. Changes in Muscle and Nerve in a Case of the Landouzy-De- 
jerine Type, 434 

115, 116. Boy with Defective Development of Scapula and Shoulder 

Muscles, 435. 436 

117. Meningocele, etc., 441 

118. Child of Seven Years with Spina Bifida and Deformity of Feet, . 442 

119. Right Hemisphere of a Simply Convoluted Brain, .... 446 

120. Mesial Aspect of same, 447 

121. Relation of Brain to Skull Lines, 44S 

122. Localization of Cortical Centres, 449 

123. Reid's Lines, 45° 

124. Motor Fibres for the Facial Nerve and Extremities, . . .451 

125. Diagram to show relative Position of the divisions of the Motor 

Tract, 452 

126. Diagram representing Motor Innervation of a Muscle, etc.. . . 453 

127. View from Before of Medulla Oblongata, etc., .... 456 

128. Course of Visual Fibres, 460 

129. Diagram representing Secondary Degeneration, etc 463 

130. Arteries at the Base of the Brain, 4.65 

131. Cortical Distribution of Middle Cerebral Artery 4' N o 



XV111 



LIST OF ILLUSTRATIONS. 



132. Distribution of Blood Supply at the Base, .... 

133. The Veins of the Du"a Mater, 

134. 135. Diagrams illustrating Aphasia, 474 

136. Case of Unilateral Nuclear Palsy, etc. Hypoglossal nucleus of left 

side, 

137. Same Case. Hypoglossal nucleus, right side, 

138. Case of Left Hemiplegia, 

139. Right Hemiplegia with Contractures and Retarded Growth of Arm 

140. Congenital Diplegia — " Frog Girl," 

141. Case of Spastic Diplegia ; cross-legged progression, 

142. Child with Congenital Diplegia ; Microcephalic Skull, Strabismus 

and Idiocy, 

143. Meningeal Hemorrhage at Birth, 

144. Cyst due to Softening of an Obstruction of Middle Cerebral Artery 

etc., 

145. Section through Portion of Motor Cortex, removed at Operation 

for Localized Epilepsy, 

146. Variously Degenerated Cells from same Section, 

147. An Old Hemorrhagic Cyst ; Tumor growing in its Walls, 

148. Section passing through Posterior Quadrigeminal Bodies, showing 

Tumor, 

149. Tumor (Sarcoma) of the Cerebellum, 

150. Vertical Section through Cyst and Tumors in Brain, 

151. Dissections showing the Guide adopted in successful Trephining 

for Abscess from Ear Disease, 

152. Patient exhibiting Symptoms of Thrombosis of Cavernous Sinus, 

153. 154. Brain with Large Anterior Defects, .... 592 

155. Large Double Porencephalic Defect, 

156. Skull of Child showing Changes four Months after Lannelongue's 

Operation, 

157. Defective Development of Occipital Lobes, .... 

158. Brain of Cerebral Type of Hereditary Spastic Paralysis, . 

1 59. Child with Pleuroplegia, 

160. Hydrocephalic Idiot 

161. Congenital Idiot, 

162. Case of Myxcedema with Idiocy, ..„.„„ 



PAGE 
467 
469 

475 

510 
5ii 

525 
527 
53i 
533 

536 

54i 

543 

544 
545 
546 

565 
567 
570 

584 
586 

593 
594 

598 
600 
602 
605 
635 
639 
645 



THE NERVOUS DISEASES OF CHILDREN. 

CHAPTER I. 

INTRODUCTION— METHODS OF EXAMINATION. 

The nervous system of the child is subject to many dis- 
eases. Some of these are identical with the nervous dis- 
orders of tne adult ; others are peculiar to the early years 
of life. 

The brain and the spinal cord do not attain their full 
development until months and years after birth, and even 
the peripheral nerves do not exhibit all their normal func- 
tions until the child is several weeks old. During this pe- 
riod of incomplete development the nervous system responds 
much more energetically to morbid influences than it does 
in later years. This is especially true of the brain. It is in 
a state of irritability and instability, and a perversion of 
functions may result from causes which would exert little 
or no influence over the nervous system of the youth or 
adult. Evidence of this is furnished by the behavior of a 
child in fever. The irregular choreiform twitchings and 
the delirium are often the outward signs of an unstable 
cerebral state, while the unusual irritability of the brain is 
proved by the occurrence of convulsions upon peripheral 
irritation to which the adult brain would not at all respond. 

In the early period of lite, too, hereditary affections of 
the nervous system are frequently manifest, and morbid 
psychic inheritance casts its shadows before. Inhibition of 
normal development may occur at any period ; family affec- 
tions are developed in the earlier years of life, and the 
acute infectious diseases of childhood are often followed bv 
serious nervous disorders. If we add to these, diseases due 
to traumatism, we have an array of nervous disorders equal 



2 THE NERVOUS DISEASES OF CHILDREN. 

to, if not in excess of, those that occur after the age of 
puberty. Childhood is exempt only from the diseases due 
to senile deterioration, from degeneration and sclerosis of 
the brain and spinal cord, and relatively free from those due 
to toxic agents, such as alcohol, metallic poisons, and syph- 
ilis ; but the effect of such diseases in the parent is exhib- 
ited with cruel persistence in the offspring. 

The diseases of the nervous system during the period of 
incomplete development are to be the special subject of 
this treatise. Before proceeding to the description of dis- 
ease it will be necessary to adopt a correct 

Method of Examination.— First of all inquire into the 
ancestry of the child. A reliable history of the physical 
and mental condition of parents, grandparents, and other 
relatives is of the greatest value in establishing a diagnosis. 
The habits and the diseases of the parents should be care- 
fully determined, for of the ills the child is heir to, not a few 
are due to alcoholism, to syphilis, to epilepsy, to hysteria, in 
one or both parents. Next to heredity, environment plays 
the most important part ; it is well, therefore, to inquire into 
the home surroundings, the manner in which the child is 
watched and cared for ; how it has been fed, trained, and 
educated. 

The previous history of the child is next in order. In 
every case inquire into the manner of its birth ; whether it 
was protracted or not ; whether or not instruments were 
used ; whether the child was asphyxiated at birth or at 
once began to breathe freely. Make inquiries regarding 
the occurrence of spasms or convulsions ; the time at which 
the child began to take notice of things, to recognize parent 
or nurse, to stand, to walk, and to talk. The occurrence of 
other diseases, of the ordinary infectious diseases of child- 
hood, of whooping-cough, of pneumonia, scarlet fever, 
measles, meningitis, should be determined, and one should 
never forget to ask whether similar nervous conditions have 
been previously observed. Then proceed to the 

Examination of the Patient. — The art of making a 
diagnosis by mere inspection has gone out of date, and is 
cried down by many ; yet I am willing to say that in fully 
one-half of the nervous diseases of children the nature of 



INTRODUCTION— METHODS OF EXAMINATION. 3 

the trouble can be suspected, if not made out, by a thor- 
ough inspection of the child without putting a finger to its 
body. I am not in favor of hurried examinations ; on the 
contrary, I wish to plead for the greatest accuracy in ex- 
amining for details ; but let the physician or student train 
his powers of observation and his diagnostic ability will be 
more acutely developed than that of the man who can never 
even suspect a disease unless he has all his tools (percus- 
sion-hammer, thermometer, aesthesiometer, electrodes) con- 
stantly at his command. 

In my lectures to students I insist that they shall study the general ap- 
pearance of a child, and should not feel satisfied until they learn to recognize 
peculiarities of facial expression, of gait, and of stature ; to distinguish be- 
tween the behavior of the normal child and the feeble-minded, between 
spastic and flaccid palsies, and to determine by the peculiar deformity of the 
foot or by the scraping noise which the patient makes in walking, which 
group of muscles is affected. It is important from the history of the patient, 
and from these general observations to get correct first impressions ; these 
first impressions are then to be confirmed by a careful detailed examination. 

Never make a diagnosis unless the child has been wholly 
undressed ; if this is not done a Pott's paralysis may be 
taken to be a traumatic myelitis, or a neuritis may be mis- 
taken for poliomyelitis anterior. Lay the child on a table 
or on another person's lap in order to get a full view of it ; 
of the relative size of head and body ; of the proportionate 
development of arms, legs, and abdomen. Remember also 
that the child has heart and lungs, liver, spleen, and intes- 
tines, which, if diseased, may hold an important relation to 
the nervous disorder present. In proceeding to a detailed 
examination it is best to begin with the head, including the 
face, then take up the upper extremities, the abdomen, and 
finally the lower extremities. 

The following scheme includes the more important points to be estab- 
lished in the examination of a child ; the exact order of inquiry is subject to 
slight modifications. 

EXAMINATION SCHEME. 

Head (Skull). — Size? Shape? Symmetrical? Dolichocephalic? Brachy- 
cephalic ? Fontanelles ? Hydrocephalus ? Bulging (Frontal or occipital) ? 
Mental condition ? Speech ? 



4 THE NERVOUS DISEASES OE CHILDREN. 

Eyes (Fundus). — Vision? Field of vision? Pupils? Light and ac- 
commodation reflexes ? Ocular movements ? Nystagmus (Lateral or ro- 
tatory) ? Is cornea sensitive ? Hearing? 

Face. — Symmetrical ? Paralysis ? Tongue ? Deglutition ? Articula- 
tion ? Sensation in face ? Teeth ? 

Upper Extremities. — Are they symmetrical ? Position ? Circumfer- 
ence of arm and forearm ? Movements (Flexion, Extension of forearm, 
wrist, fingers) ? Paralysis ? Tonus of muscles ? Are muscles atrophied or 
hypertrophied ? Reflexes ? Contractures ? Electrical reactions ? Sensa- 
tion ? 

Trunk. — Respiration ? Sensation ? Reflexes (Abdominal, Epigastric, 
Cremasteric) ? Action of muscles ? 

Lower Extremities. — Are they symmetrical ? Circumference of thighs 
and calves ? Ability to stand ? Romberg's symptom ? Ability to walk ? 
Gait (Paretic, Spastic- paretic, Ataxic, Cerebellar) ? Movements of individual 
groups of muscles ? Is child able to raise thigh ? To flex and extend 
thighs, legs, toes ? To stand on tiptoes ? To elevate toes, keeping heels on 
ground ? Are muscles paretic or paralyzed, atrophied or hypertrophied ? 
Electrical reactions ? Reflexes (Knee-jerks, Ankle clonus, Achilles tendon 
reflex) ? Contractures ? Sensation ? 

Vesical and Rectal Reflexes? 



Examination of the head of a child often gives us val- 
uable information. The normal head should be well 
rounded and symmetrical. According to the age of the 
child the size will vary. The average horizontal circum- 
ference at birth (measured by a line passing from the gla- 
bella around the occipital protuberance) is between 38 and 
42 ctm. ; at the end of one year between 45 and 52 ctm., 
and in later years it may grow gradually to 56 ctm. Any 
marked departure from these measurements is abnormal, 
but heads of tolerable size may be associated with deficient 
development of parts of the brain. I have seen cases with 
normal circumference in which the anterior defect was not 
evident in the measurement in consequence of a slightly ex- 
cessive development of the occiput. A normal circumfer- 
ence is also present at times, although the actual cranial ca- 
pacity may be very much diminished by a receding frontal 
bone. 

The following table will give the chief measurements of the skull in chil- 
dren ; a few centimetres should be deducted for the thickness of hair and 
scalp. 



INTRODUCTION— METHODS OF EXAMINATION. 
Table of Cranial Measurements in Children. 





New-born. 


End of 1st yr. 


1st to 7th yr. 


10th year. 






M. 


F. 


M. 


F. M, F. 


M. 


47 




i. Circumfer- 
ence 

2. Binauricu- 


34- 

20.0 
385 to 450 


34.o 
20.0 


42.0 

25- S 
700 to 1, 000 

28.0 


42.0 
25.0 


34 to 46 ... . 
27 


49 


Taken around 
glabella and 
occipital pro- 
tuberance. 


3. Volume . .. 

4. Naso- occi- 

pital arc. 


j B to opposite 
ext. aud. mea- 
tus. 
1,300 ... Volume is to cir- 


i 
20.0 22. a 


28.0 
10.0 
10. 










cumference as 
1,350 is to 50 
(in the adult). 

N, 0, T. 

N, 0. 


5. Naso-breg- 
matic arc. 


7-7 
9.0 


7-7 

9-o 






12 


6 Bregmatic 
lamb, arc. 






12 




e to A. 








1 




Fig. 1. — Craniometrical Lines. (Benedict and Peterson.) 

The formula for the cephalic index is length : breadth :: 100 : x. An in- 
dex below 78 is dolichocephalic ; 78 to 80 mesocephalic ; above So brachyce- 
phalic. The facial length is determined by a line passing from N to lowest 
part of chin. 

Both halves of the head should be symmetrical. Asym- 
metry occurs chiefly in connection with defective develop- 



6 



THE NERVOUS DISEASES OF CHILDREN. 




Fig. 2 

Six Years. 
(Peterson.) 



ment of the brain and with early cerebral lesions. (Fig. 
2.) The chief abnormalities of skull formation are as fol- 
lows: Dolichocephalus, a long skull, the anterior poste- 
rior diameter being pro- 
portionately greater than 
the transverse. Many new- 
born children are dolicho- 
cephalic as the result of 
compression of the head 
in the pelvic canal, but af- 
ter a few days or weeks 
the head is well rounded. 

Asymmetry of Skull in a Male, aged Brachycephalic — the skull 

Right Hemiplegia from Birth. j s s hort in the antero-pos- 

terior diameter. The terms 
microcephalus and macrocephalus need no further expla- 
nation. * 

Bulging of the frontal or occipital bones is important 
as an indication of hydrocephalus. If there is a very con- 
siderable increase of intracranial fluid the sutures may 
be pushed asunder and can be felt distinctly through the 
scalp. This same condition occurs in some cases of neo- 
plasm. In passing the hand over the head the fontanelles 
can be felt. The occipital fontanelle should be closed after 
a few weeks, the anterior remains open until the tenth or 
twelfth month. If it closes long before this period there is 
premature ossification of the sutures ; if it remains open 
much longer, it is a certain sign of rickets. 

After the examination of the head Ave may pass at once 
to the inquiry into the mental condition of the child. Ac- 
cording to its age we must ask whether it recognizes 
its mother or nurse; whether it has learned to play, to 
understand what is said to it ; whether it begins to imi- 
tate sounds, to articulate, etc. ; in short, whether it shows a 
normal awakening of the mind. In children a little more 
advanced in years it is necessary to determine whether the 
child is able to keep up with others of its age ; whether it 



* A few special terms have been in use for oddly shaped heads : Keel-shaped skull, 
scaphocephalus ; triangular skull, trigonocephalus ; steeple-shaped skull, oxycephalus ; 
obliquely deformed skull, plagiocephalus. 






INTRODUCTION— METHODS OF EXAMINATION. 7 

has been able to acquire the ordinary rudiments of knowl- 
edge. The physician should assure himself of these points 
by a personal examination, and should not depend upon the 
statements of parents and relatives. 

A few extracts from Preyer's observations on his own 
child will show what may be expected of a normal child at 
different stages of its development: 

During First Month. — Recognizes difference between light and 
dark objects (even on first day) ; follows with its eyes object moved slowly 
before it (as early as eleventh day) ; begins to hear about the fourth day ; 
recognizes sounds toward end of first month ; learns to distinguish between 
bitter and sweet ; recognizes disagreeable odors ; first tears on twenty-third 
day during a crying spell ; expresses displeasure by turning head away, by 
shutting its eyes, and, of course, by crying ; begins to smile. 

During Second Month. — Recognizes human voices and direction 
from which sound comes ; turns head toward low sounds ; is quieted by 
song ; smiles when music is heard ; recognizes its mother. 

During Third Month. — Moves arms, expressive of pleasure ; listens 
attentively ; is able to support head a little ; uses definite sounds in crying. 

During Fourth Month. — Associated eye movements perfect ; stares 
at new objects ; recognizes strange surroundings ; reaches after distant ob- 
jects ; first attempt to sit upright. 

During Fifth Month. — Recognizes strangers as such ; likes to take 
hold of everything ; stretches out its arms to be taken up ; holds head 
straight ; sits alone ; moves legs as if to walk ; forms syllables. 

During Sixth Month. — Distinguishes faces ; stares at strangers ; 
smiles, if smiled at ; smiles with relatives, not with strangers ; turns its head 
toward a person leaving the room ; begins to creep ; " crows." 

During Seventh Month. — Follows objects dropping out of its 
hands ; recognizes its image in mirror with evident pleasure ; points with 
finger at pictures ; purposive movements ; associates persons and names ; 
extends hand when asked ; articulates a number of different sounds in cry- 
ing and in " lolling to itself." 

During Eighth Month. — Sits upright when it is carried ; some chil- 
dren attempt to stand and to walk. 

During Ninth Month. — Begins to imitate tunes ; laughs heartily ; 
begins to beg for things. 

During Tenth Month. — Takes an intense interest in its food ; rec- 
ognizes parent after absence of several days ; he begins to walk alone ; an- 
swers questions by motions and indicates where certain things are. 

During Eleventh Month.— Stands quite alone ; pushes chairs ; 
makes first attempt to repeat sounds impressed upon its mind ; begins to 
articulate its own name ; understands language fairly well. 

During Twelfth Month.— Imitates laughter of others ; stretches its 



8 



THE NERVOUS DISEASES OF CHILDREN. 



arms out to enforce its demands ; improvement in walking and standing ; 
looks at others attentively while they eat. 

During Fourteenth, Fifteenth, and Sixteenth Months. — In- 
dependent speech is acquired, and repeats spoken words easily ; in seven- 
teenth month may speak short sentences, using verbs ; from this time on 
there is steady improvement in memory of words and use of language. 

At two years child may learn to repeat rhymes, to detect colors, etc. 

If the mental condition of the child has been satisfac- 
torily determined, the special senses should be examined. 
" Does the child see? " is a question often answered affirma- 
tively by the mother, when a closer examination proves 
that the child is totally blind. Mothers are easily deceived 
in this, for the restless movements of the eyes in young 
children are supposed to be purposive and part of the vis- 
ual act. To test vision use a candle or a taper, and pass it 
in front of the eyes at some distance from the head, so as 
to avoid heat sensations, and note whether the child fol- 
lows the light. Do not be misled by accidental movements 
and avoid using rattles, for the child may turn its eyes in 



OD 




Grasset et 



Fig. 3.— Normal Visual Fields for Colors. 



the direction from which the sound issues without seeing 
the object. Use also simple substances of different colors 
(pencils, papers, glass, etc.), and see whether the child fol- 
lows these objects. 

It is a matter of still greater difficulty to test the field 



INTRODUCTION— METHODS OF EXAMINATION. 9 

of vision. In very young children it may be altogether 
impossible, but after a child has reached the age of five 
months or thereabouts, it may be possible to make a rough 
test of the visual field by passing objects from above and 
below, as well as from the sides, into the visual field, and 
noticing when the child begins to turn its eyes toward this 





Fig. 5.— Field of Vision in a Case of Left-sided Hemianopsia. The Shading Repre- 
sents the Blind Part ; the Oval Outline is the Average Normal Field. (Gowers.) 

object. The ordinary test as applied in the adult cannot 
be employed for children until they have developed con- 
siderable intelligence. 

The condition of the visual field is often overlooked and the defects re- 
ferred to are much more frequent than they are generally supposed to be. 
An examination should be made in all cases of cerebral palsies, and in those 
in which cerebral tumor is suspected. 

In cases in which a very complex examination seems desirable, and these 
are relatively few, special tests should be made for form and color. The 
hemianopsic defects as they occur in tumors, and in cerebral palsies (first 
described by Freud), the concentric limitations occurring in hysteria, are 
the most common defects in children. 

The pupils are to be tested for their size and power of 
contractility ; both should be equal and contract promptly 
to light and during accommodation. The movements of 
the ocular muscles will be best understood by reference to 
the subjoined table. Conjugate deviation is due to disease 
in the hemisphere, and according to Grasset to lesions in 
the first and second frontal convolutions and in the angular 
gyrus and its vicinity ; the patient looks toward the lesion 
except in the case of spasm of the muscles, when the patient 
looks away from the lesion. Nystagmus occurs in many 
cerebral affections, particularly in cases of early cerebral 
disease or congenital defect. It is seen also in cases in 



IO 



THE NERVOUS DISEASES OF CHILDREN. 



which there are stigmata of degeneration as well as in 
multiple sclerosis. In the latter it may be bilateral or 
rotatory. The corneal reflexes (the prompt closure of the 
eyelids if the cornea is touched carefully with the head of 
a pin or a small lead-pencil) should be tested. In a great 
many individuals this reflex does not ensue upon irritation 
of the conjunctiva, a fact that has deceived many physi- 
cians. 

Muscles of the Eyes and Face. 











Diseases in 


Name of Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle 


Muscle. Function. 


cient Action. 


by 


in 


is commonly 
Involved. 


Ciliary . . . Makes 1 e n s e 


Loss of accommo- 


The thirdCorpora 


l"gS.c;S° 


more convex ; 


dation ; spasm of 


nerve. 


quadrigem- 


3.2S M 

3 - c? = 


aids in accom- 


ciliary muscle may 




ina and pe- 


modation. 


cause myopia. 




duncle. 


Sphincter Contraction of 


Paralytic mydria- 


The thirdCorpora 


P.^o-5 « 


iridis. 


pupil to light 


sis ; no contrac- 


nerve. 


quadrigem- 


^»° V ■ — 




and during 


tion to light and 




ina and pe- 


.2 S, h o & 




accommoda- 


during conver- 




duncle. 


i go |-S| 




tion. 


gence or accom- 










modation. 






tmu 
theri 
in ; ti 
rior 
tary 


Dilatato r 


Dilates pupils; Pupil does not re- 


Sympathetic 




pupillse. 


as a result of 


spond to sensory 






•-,£3 5 « — 

« ~ • ft - 




sensory or 


stimulation. 






"S .~ ~ — u 




psychic stim- 








C .12 r, wS ■" 




ulation. 








Meni 
eye 
ofm 

lis ; 
drug 


Rectus su- 


Moves eye up- 


Upward movement 


The third 


Corpora 


, •->, 


perior. 


ward and in- 


limited; diplopia; 


nerve. 


quadrigem- 


.2 js 




ward. Acts 


false image 




ina and pe- 




with inferior 


above ; deficient 




duncle. 


O 3 
ft_a 




oblique. 


rotation of eye- 
ball. 






"C ft 


Rectus in- 


Moves eye in- 


Strabismus diver- 


As above. 


Corpora 




ternus. 


ward. 


gens ; defective 




quadrigem- 


13 w 






inward pull. 




ina and pe- 
duncle. 


c 3 • 

2 o o 

a s ea 


Rectus in- 


Moves eye 


Imperfect move- 


As above. 


Corpora 


ferior. 


downward 


ment downward ; 




quadngem- 


•2o S 




and rotates it 


eye rotated out- 




ina and pe- 


g.S-'-S 




inward. Acts 


ward. 




duncle. 


! --"3 rt 




with superior 








I S- 3 CJ 

O C 7? 




oblique. 










Obliquus 


Acts with rec- 


Imperfect move- 


As above. 


Corpora 


inferior. 


tus superior, 


ment upward ; eye 




quadrigem- 


: •-" "rt 




moving eye 


rotated inward. 




ina and pe- 


5 8*S 




upward and 






duncle. 


■a^a 




outward, and 








\ Z~ s 




rotates it up- 








,- rt O 
— t- y 




ward. 








g-S- 


Obliquus 


Moves eye On looking down- 


The fourth 


Peduncle 


— u - — . 


superior. 


downward 


ward eye is pulled 


nerve (tro- 


near the 






and outward. 


inward ; conver- 


chlearis). 


corpora| 


£ --^ 




Acts with in- 


gent strabismus ; 




quadrigem- 


.£-s| 




ferior rectus ; 


diplopia on step- 




ina. 


m .5 

g3 5 n 
2 §.2 




rotates down- 


ping downward. 








ward. 


















LQ 5rt 



INTRODUCTION — METHODS OF EXAMINATION. 
Muscles of the Eyes and Face. — Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle 


Muscle. 


Function. 


cient Action. 


by 


in 


is commonly 
Involved. 


Rectus ex- 


Moves eye out- Out ward move^The sixth 


Pons 


As before, and 


ternus. 


ward. 


ment impaired ; 


nerve (ab- 




in disease of 






head turned in di- 


ducens). 




the pons. 






rection of para- 












lyzed muscle. 








Levator 


Raises upper 


Ptosis ; eye closed; 


The third 


Pedun c le 


Associated 


palpebrae 


eyelid. 


may be opened a 


nerve. 


and cor- 


with other 


superioris 




little by frontalis 




pora quad- 


third nerve 






muscle. 




rigemina. 


diseases; often 
congenital. 


Orbicular- 


Closes eyelids. 


Eyes cannot be 


The seventh 


Pons 


Peripheral fa- 


is palpe- 




closed (lagoph- 


nerve. 




cial neuritis ; 


brarum. 




thalmos). 






affections at 
base of brain 
(meningi t i s , 
tumor, and 
the like) ; in 
some dystro- 
phies. 


Fronta 1 i s 


Raises eye- 


Imperfect raising 


As above. 


As above. 


As above. 


and cor- 


brows ; fold- 


of eyebrows ; no 








Teg a t o r 


ing of skin of 


frowning ; lines of 








supercilii 


forehead, as 
in frowning. 


forehead d i s a p- 
pear. 








Orbicula- 


Move lips and 


Face distorted and 


The seventh 


Pons 


Cerebral apo- 


ris oris, 


cheeks, as in 


pulled toward 


nerve. 




plexies (es- 


buccin- 


speaking, kiss- 


healthy side ; ina- 






cape of upper 


ator, and 


ing, showing 


bility to pout lips 






branches of 


other 


of teeth, and 


or to whistle; drib- 






fa c i a 1 ) ; pe- 


muse 1 e s 


the like. 


bling of saliva 






ripheral facial 


of face. 




from paralyzed 
side ; flapping of 
cheek with each 
expiration, owing 
to paralysis of 
buccinator ; d i s- 
app earanceof 
nasolabial fold. 






palsy (of t e n 
due to expos- 
ure ) ; in all 
lesions of 
pons ; in dys- 
trophies. 


Masseter , 


Masseter, tem- 


Mastication imper- 


The fifth 


Pons 


Rarely affect- 


tempor- 


poral, and in- 


fect ; if in spasm, 


nerve. 




ed in children 


al, and 


ternal ptery- 


jaws cannot be 






except in tet- 


ptery- 


goid elevate 


opened; jaw 






anus ; in facial 


goids. 


lower jaw; 


moved toward 






hemiatrophy; 




external pter- 


paralyzed side by 






in lesions 




ygoid draws 


action of ptery- 






of pons. 




jaw forward ; 


goids. 










the two ptery- 












goid muscles 












of one side 












acting togeth- 












er move teeth 












toward oppo- 












site side. 











Mydriasis can be caused by paralysis of sphincter iridis (third nerve), or 
by spasm of the dilator muscles (mydriasis spastica). Atropin has a simi- 
lar effect by paralyzing the sphincter, and contracting the dilator. 



12 THE NERVOUS DISEASES OF CHILDREN. 

Myosis may be due to irritation of sphincter supplied by the third nerve, 
and occurs in early stages of many cerebral affections, including apoplexy ; 
or it may be due to paralysis of the dilator muscle. 

Inequality of pupils is sometimes congenital, more often due, however, to 
cerebro-spinal disease (meningitis, syphilis, multiple sclerosis, hereditary 
ataxia). Muscles of both eyes act conjointly. If such action is deficient we 
speak of conjugated paralysis. There may be paralysis of lateral, upward or 
downward movements, without total palsy of any one muscle. If individ- 
ual muscles are paralyzed, or muscles in one eye only, double vision is certain 
to result. 

In diplopia the exact position of true and false images will help to de- 
termine the degree of ocular paralysis. If one muscle is paralyzed second- 
ary deviation of the associated muscle in the other eye may take place. 

Paralysis of the inner muscles of the eye is designated as ophthalmo- 
plegia interna ; paralysis of the muscles moving the eyeball, as ophthalmo- 
plegia externa, and such ophthalmoplegia may be total or partial. Total 
ophthalmoplegia externa and interna is the occasional accompaniment of dis- 
ease at the base ; partial ophthalmoplegia (only part of the muscles sup- 
plied by the third nerve, for instance, being affected) points to nuclear disease, 
but syphilitic infiltration of the root fibres may simulate nuclear disease. 

Narrowing of the orbital fissure (sometimes congenital) may be due to 
paralysis of smooth fibres in the lid, innervated by the sympathetic. Clonic 
movements of eye muscles we term nystagmus ; this may be lateral or rota- 
tory. 

An examination of the sense of hearing is often called 
for. In children with defective development this may be 
entirely wanting. The simplest tests are to clap the hands 
at a distance from the child's head, to whisper its name, to 
use a loudly ticking watch — even very young children will 
be attracted by the sound if it is perceived. In the case of 
older children tuning-forks may be applied to the head. In 
disease of the ear proper the vibrations would be perceived, 
but not so if the nerve itself, the labyrinth, or the auditory 
tract were involved. 

In continuing the examination of the head we must note 
whether the face is symmetrical. Asymmetry may point 
to congenital defect or to a preceding palsy. It is of some 
interest to know that asymmetry of the face and of the nose, 
adhesion of the ear-lap, malformations of the outer ear, the 
existence of a torus palatinus (felt as a ridge in the roof of 
the mouth) are the ordinary stigmata of a degenerative 
type, of which so much has been made by the Italian crimi- 
nologists of the present day. If such stigmata prove a 



INTRODUCTION — METHODS OF EXAMINATION. 



13 



predisposition to the development of nervous troubles, to 
crime, or to insanity, it is well that the physician should 
know of them. 

The teeth play a very important part in the life of every child. If not de- 
veloped between the ages of six months and one year, rickets must be 
suspected. Notched teeth are suspicious of syphilis. Unusually early de- 
velopment of the teeth is an evidence of premature ossification. 

Muscles of Tongue, Palate, and Pharynx. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Genio- 
glossus. 

Styloglos- 
sus. 



Li ngual 
muscle 
proper. 



Az y g o s 

uvulse. 



L e vat o r 

palati. 



P a 1 a t o- 

pharyn- 
geal mus- 
cles. 



Stylo-pha- 
ryngeus. 



Pushes tongue 
to opposite 
side. 

Raises tongue 
backward 
and upward. 



All movements 
of the tongue 
itself. 



Shortening of 
uvula. 



Raises the vel- 
um palati. 



Prevent food 
from passing 1 
toward up-| 
per part of 
pharynx and| 
posterior' 
nares. 

Helps to draw. 
larynx up- 
ward so as to 
be closed by; 
epiglottis and; 
ov ertopp e d 1 
by tongue. 



Tongue when pro- 
truded deviates to 
paralyzed side. 

Tongue cannot be 
moved backward 
or hollowed out 
(action deficient 
in many healthy 
subjects). 

When lying in 
mouth deviation 
to healthy side ; 
when protruded 
deviates to paral- 
yzed side ; if one 
or both halves are 
atrophied tongue 
looks shrivelled. 

Uvula deviates to- 
ward sound side ; 
if both sides are 
paralyzed there is 
nasal tone and 
regu r g i t a t i o n 
through nose. 

Arch cannot be 
raised in the in- 
tonation of " ah ; " 
if paralysis is bi- 
lateral flapping of 
arch and regurgi- 
tation of food 
through nose. 

Regurgitation of 
food; nasal 
speech. 



Imperfect degluti- 
tion ; food gets 
into windpipe. 



Innervated 
by 



Represented 



The twelfth Medulla, 
nerve (hy- 
poglossal). 

The twelfth Medulla . 



The twelfth Medulla, 
nerve. 



Probably Medulla 
pharyngeal 
plexus; 
seventh 
nerve (?). 



As above. 



Medulla. 



The fifth Pons 
nerve. 



Glosso -pha- Medulla, 
ryngeal. 



Diseases in 
which Muscle is 
commonly In- 
volved. 



Bulbar pal- 
sies (acute 
and chron- 
ic) ; in spe- 
cific and tu- 
ber c ul ar 
diseases of 
base ; dys- 
r o p h i e s 
(rare). 



As above 



As above ; see 
also seventh 
nerve affec- 
tions. 



Basilar 

tions. 



affec- 



Bulbar affec- 
tions and dis- 
eases of the 
base. 



H 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Tongue, Palate, and Pharynx.— Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient Action. 


by 


in 


commonly In- 
volved. 


Constric- 


Help to push 


Food is swallowed 


P h a ryngeal 


Medulla 


Diseases of the 


tors of 


food into gul-l very imperfectly ; 


plexus. 




base (bulbar). 


pharynx. 


let. sticks in throat. 








Laryngeal 


Movements of Hoarseness and Recurrent 


Medulla .... 


Bulbar troub- 


muscles. 


vocal cords in 


difficulty in 


laryngeal 




les (similar 




res pirat io n 


breathing ; laryn- 


nerve ex- 




symptoms 




and in articu- 


goscopy examina- 


cepting the 




maybe caused 




lation. 


tion reveals false 


crico- thy- 




by tumors and 






position of vocal 


roid mus- 




foreign bodies 






cords (see special 


cle. 




in larynx). 






text-books). 









The movements of the tongue have been considered in the annexed table. 

A short frenulum may cause difficulty in articulation, but it is often suspected 

to be the cause in cases in which the defect is due to a distinct cerebral 

lesion. 

Sensory disturbances of the face are rare in children, but, if suspected, 

tests should be made carefully with cotton, with the head and point of a pin, 

and by application of hot and cold ob- 
jects. Subjective sensory disturbances 
(neuralgia) may occur as in adults, and 
will vary in the distribution according 
to the branches affected. (Fig. 6.) 

In examining- the trunk and 
the four extremities no fact is 
more important to establish 
than the existence of paresis, 
or paralysis of individual mus- 
cles, or of groups of muscles. 
To be able to do this the ex- 
aminer must know the action 
of all the more important mus- 
cles in health and the disturb- 
ances due to disease of such 
muscles. These are the very 
facts in which the student's knowledge is, as a rule, most 
defective. In the appended tables I shall endeavor to 
give the principal points in concise fashion : 




Fig. 



6. — Sensory Chart of Face. 
(C. S. Freund.) 



INTRODUCTION— METHODS OF EXAMINATION. 



15 



Muscles of Head and Neck. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



mastoid. 



S t ern o- R ai s e s and Inability to raise 
c 1 e i d o- turns face toj head from bed, or 
opposite side; other horizontal 
head inclines position, if both 
to same side; muscles are affect- 
if both mus- ed ; if one muscle 
cles act con-j is affected, no 
jointly head is marked change of 
brought for- position, unless 
ward. opposite . muscle 

is contractured ; 
spasm of muscle 
frequent ; head in- 
clined to one side 
R e c t u s To flex head. Cannot flex head 1 
capitis so as to bring 

antic us chin on chest, 

major. 



R e c t u s To flex head, 
capitis 
anticus 
minor. 

R e c t u s 1 Slight rota 



Innervated 
by 



capitis 
lateralis. 



tion. 



Seal eni ] Elevate ribs 



anterior 
medi- 
u s , e t 
po s t e- 
rior. 



L o ngus 
colli. 



when verte- 
bral column 
is fixed ; aid 
in inspira- 
tion ; slight 
lateral flex- 
ion. 

Flexor of ver- 
t e b r a 1 col- 
umn. 



Deficient rota- 
tion scarcely 
noticeable, un- 
less sterno-clei- 
do-mastoids are 
diseased. 

Deficient inspi- 
ratory move- 
ments. 



Imperfect flexion 
of upper spine. 



Spinal ac- 
cessory. 



Upper cervi 
cal. 



Represented 



Diseases in 
which Muscle is 
commonly In- 
volved. 



Medulla and In bulbar and 



second and 
third cer- 
vical seg 
ments. 



Lower cervi- 
cal nerves. 



Lower cervi- 
cal nerves. 



Upper cer- 
vical seg 
ments. 



Lower cervi- 
c a 1 seg- 
ments. 



cervical cord 
affections ; in 
later stages of 
p r o g r essive 
muscular 
a t r o p h i es ; 
o c c asionally 
in neuritis. 



Diseases of 
the cervical 
region (my- 
elitis, men- 
i n g i t is, 
tumor ; pro- 
gr e s s i ve 
wasting of 
muscles. 



The sterno-cleido-mastoid and the other muscles of the head and neck, 
mentioned in these two tables, are more frequently the seat of spasm than of 
paralysis. The spasm may be restricted to one muscle or may involve 
several. It may be the result of organic disease in the medulla oblongata 
or spinal cord. More often it is functional in character and related to other 
spasmodic conditions. The same group of muscles may be involved in the 
ordinary rheumatic affections (torticollis, caput obstipum). 



1 6 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Shoulders and Upper Extremity. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Innervated 
by 



Trapezius. 
i. CI a -J 
v i c u 1 ar 
portion 
(respi- 
ratory ; 
outer 
third of; 
c lavicle 
to occipi- 
tal bone). | 



Pulls head 

backward; ro-| 
tates slightly 
toward side ot 
muscle , so 
that chin is 1 
turned to op- 
posite side ; 
contraction ot 
both clavicu- 
lar portions, 
bends head 
backward; 
slight eleva- 
tion of shoul- 
ders ; aids in 
deep inspira- 
tion. 



portion; der - blade ; 
(fro m elevation of 
acromion acromion 
and outer (clavicle goes 
spine of along), 
scapula to! 
ligament. | 
nucha 
and up- 
per dorsal 
spines). I 

3. Low e r Adduction ol 
portion scapula to- 



Deficient back- 
ward movement 
of head; not 
marked as rule 
because deep 
muscles perform 
this function ; 
shoulder does not 
move during in- 
spiration. 



Spinal ac 
cessory. 



I Diseases in 
Represented , which Muscle is 
m ! commonly In- 

volved. 



2. Middle Raises shoul- Acromion depress- Spinal ac- 

cessory 
nerve. 



and ad- 
ductor. 



R h o m 
boids. 



ward median 
line. 



Oblique move- 
| ment of scap- 
I ula from be- 
low, upward 
and inward, 
so that infe-i 
rior angle is 
brought near- 
er the medi-j 
an line ; hold 
spinal margin 
of scapula 
down to tho- 
rax. 



ed by weight ot 
upper extremity ; 
inner upper an- 
gle may be pulled 
upward by levator 
anguli scapulae ; 
internal lower an- 
gle is nearer to 
median line. 



Margin of scapula Spinal ac- Medulla 



MedullaProgre ssive 
and second muscular 



and third 
cervical 
segments 



As above. 



is about ten ctm 
distant, instead of 
being five or six 
ctm. distant from 
median line ; loss 
of adductor may 
be covered up by 
action of rhom- 
boids ; rounding 
of back. (Fig. 8.) 

Deep groove be- 
tween inner mar-j 
gin of scapula and 
thorax ; if serra- 
tus is normal, this 
groove disap- 
pears if arm is ex-! 
tended forward ; 
shoulder -blade 
cannot be approx- 
imated to median 
line. (According 
to Duchenne this 
can be affected by 
upper portion of 
latissimus dorsi.) 



cessory 
nerve. 



Fifth cervi- 
cal. 



wasting ; dis- 
eases of me- 
dulla and up- 
per cervical 
cord; clavi- 
cular portion 
least fre- 
quently in- 
volved. 



As above. 



and second 
and third 
cervical 
segments. 



Fourth and As above. 
Fifth cervi- 
c al seg- 
ments. 



INTRODUCTION-— METHODS OF EXAMINATION. 



17 



Frontalis 

Facial Nerve 

Corrugator Supercilii 

Orbicular. 

Palpebr. 

Muscles of Nose 

Zygomatic! 

Orbicularis Oris 

Median Division of ) 

Facial Nerve \ 

Masseter 

Levator Menti 

Hypoglossal Nerve 

Lower Facial Nerve 

Platysma Myoides 

Hyoid Muscles 



Omohyoid 



Anterior Thoracic Sup- ) 
plying Pectoral Mus- v 
cles ) 




( Region of Central 
( Convolutions 



Speech Area 
Temporal 

Facial 

Trunk of Facial Nerve 

( Posterior Auricular 

\ Nerve 
Facial 

Lower Branch 
Splenius 

Sternocleido Mastoid 
Spinal Accessory Nerve 
Levator Anguli Scapulae 

Trapezius 
Scapular Branch 

Circumflex Nerve 
PosteriorThoracicNerve 



Phrenic Nerve Erb's Point Brachial Plexus 

Fig. 7.— Motor Points of Face. (Erb.) 




Fig. 8.— Patient with Hypertrophy of Infra and Supra Spinati, Showing Rotation oi 
Right Scapula and Deep Groove along Inner Margin of Scapula (Atrophj of 
Rhomboids and Slight Atrophy of Lower Portion of Trapezius). 
2 



1 8 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Shoulders and Upper Extremity. — Continued. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Innervated 
by 



Levator Draws superi- Isolated 
a n g u 1 i or inner angle rare, 
scapulae. ! of scapula up- 
\ ward ; aids 
in shrugging 
of shoulders. 



Represented 



Diseases in 
which Muscle is 
commonly In- 
volved. 



paralysis Third and Second and D y s t r o phies 



Serr a t u s Rotation o t 



Fifth cervi 
cal nerves, 



magnus. 



Scapula pulled up- Poster i or 
ward ; lower inner thoracic 
angle nearer the nerve 
median line ; armj 
cannot be raised 
above horizontal 
position ; if arm is 
stretched forward 
scapula is re- 
moved from tho- 
rax ( " winged 
scapula ") ; dur- 
ing abduction of 
arm, scapula is 
moved nearer to 
median line, and 
crowds trapezius 
and rhomboids 
forward. 



D e 1 1 o i d To raise arm Can raise shoulder Circumflex. 



shoulder- 
blade out- 
ward , and 
slight eleva- 
tion of acro- 
mion ; holds 
inner margin 
of scapula to 
thorax; brings 
arm from hor- 
izontal to ver- 
tical position. 



(three di- to horizontal 



visions). 



Infraspi- 1 
natus. ! 

Teres j 
minor. J 

Sub scap- 

ularis. 



S u praspi- 
natus. 



position, and 
forward, out- 
ward, or back- 
ward ; move- 
ments possi- 
ble only if 
s c ap u 1 a is 
fixed by ac-' 
tion of serra- 
tus and tra- 
pezius. 

Rotator hu- 
meri posticus 
(Duchenne) ; 
rotate arm 
outward. 

Rotator hume- 
ri a n t i c u s 
(Duchenne) ; 
rotates arm 
inward. 

Helps to 
steady shoul- 
der-joint and 
to elevate 
arm forward 
and outward ; 
outer angle of 
scapula is de- 
pressed. 



but not arm; 

shoulder flattened 
(atrophy) ; groove 
between acromion 
and head of hu- 
merus ; each di- 
vision of deltoid 
may be paralyzed 
singly. 



Arm cannot be S u p r , a 
moved outward. _ sca P^r, 

Difficulty in writ 
ing (Duchenne). 

Arm cannot be Subscapu- 
moved inward ; lar nerve, 
scapula is rubbed 
against ribs. 

According to Du- Sup rascap- 



C ircum- 

flex. 



Fourth (?) and cervical 
c e r v i c a 1 diseases. 
segments. 



Fifth an d Progressive 
sixth cervi- muscular at- 



c al s eg 
ments. 



rophies (dys- 
trophies) ; 
ne u r i t i s of 
part of the 
brachial plex- 
us; after trau- 
matic injuries 
to shoulder ; 
i n cervical 
cord affec- 
tions. 



Fourth, fifth, As above; also 
and sixth in Erb's form 
cervical of obstetrical 
segments. paralysis. 



Fourth, fifth, 
j and sixth 
cervical 
segments. 



chenne, humerus 
is separated still 
further from acro- 
mion, if supra- 
spinal is affect- 
ed in addition to 
deltoid. 



ular. 



Fourth cer- 
vical. 



As in case of 

deltoid. 



As above. 



INTRODUCTION- — METHODS OF EXAMINATION. 



19 




h U s 



■ ■ ^B^ 








\£W 




• • ■ H^^^^^^^^H ■ ■ ^^r 




& ^^\ 




> WK awik 41* \ % * 


A - M 


MHr YtwlnMm^m"' 




*&tt^L: 




■ Wk ■^ : %m m 




Kg M 


^ ma ^ mm ^ ^^B 



be 

.s • 

S ss 

&o 

£ S3 
to o 

Q '53 

o 

a a 
^ *-■ 

« < 

"55 ^3 

1/5 a 

£ rf 

2 8 

S: * 

10 o 

Sir 

On 






20 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of Shoulders and Upper Extremity. — Continued. 











Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated j Represented 


which Muscle is 


Muscle. 


Function. 


cient Action. 


by in 


1 commonly In- 
volved. 


L a t i ssi- 


Pulls the arm, 


Arm cannot be 


S u b s c apu- Sixth a n d As in progres- 


mus dor- 


when raised, 


moved backward ; 


1 a r , also seventh cer- 


sive atrophies 


si. 


downward 


insufficient exten- 


branches of vical. 


and dystro- 




and back- 


sion of dorsal 


dorsal and 


phies ; in cer- 




ward ; if arm 


spine ; trunk can- 


lumbar 


vico-dorsal 




is at rest up- 


not be moved lat- 


nerves pass- 


lesions; in 




p e r portion 


erally. 


ing through' 


neuritis. 




brings scap- 




muscle. 






u 1 a nearer 










the median 




■ 






line ; united 










action of up- 










per third of 










both muscles 










causes exten- 










sion of dorsal 










trunk ; single 










action causes 












lateral move- 












ment of trunk. 










Teres ma- 


Rotates raised 


Very few symp- 


S u b s c apu- Seventh cer- 


As above. 


jor. 


humerus in- 
ward; adduc- 
tion of arm to 
thorax ; slight 
elevation of 
shoulder. 


toms ; action sup- 
plied by other 
muscles. 


lar. 


vical. 




Pectoralis 


Clavicular 


Imperfect adduc- 


Anterior 


Fifth, sixth, 


Amyotrophies 


major. 


portion de- 


tion of arm ; par- 


thoracic. 


and seventh 


and dystro- 




presses hu- 


alysis can be dis- 




cervical. 


phies, chiefly; 




merus from 


covered best by 






also in lesions 




raised posi- 


extending arms 






of brachial 




tion to hori- 


and tryingto press 






plexus. 




zontal ; ad- 


volar surfaces 










du ct i on of 


again s t each 










arm, as in 


other. 










giving a bless- 












ing ; sternal 












p o r t i on de- 












presses arm 












c o m p letely, 












and if arm is 












at rest draws 












acromion for- 












w a r d and 












backward. 






1 
1 


m 



Absolute paralysis of both upper extremities is rare in children. If an 
entire extremity is paralyzed, the arm either hangs limp by the side of the 
body, or is flexed at the elbow. If the arm is lifted it falls by its own weight. 
Children sometimes refuse to make an effort, or do not understand what is 
wanted of them. After a few trials it is generally possible to decide whether 
there is a loss of power or mere lack of effort. Total paralysis of the upper 
extremity occurs either from disease of the brachial plexus, from spinal cord 
affections, or from cerebral lesions. If due to the first or second causes, the 



INTRODUCTION—METHODS OF EXAMINATION. 21 




Fig. ii.— Young Boy with Multiple Neuritis, showing Double "Wrist Drop" and 

Slight " Foot Drop." 




Fig. 12. — Appearance of Hand in an Early Stage of Progressive Muscular Atrophy 
Atrophy of Abductor Brevis and Opponens Pollicis. (Duchenne. ) 



22 



THE NERVOUS DISEASES OF CHILDREN. 



paralysis is flaccid ; if due to the third cause, the paralysis is spastic, and 
very often part of a hemiplegia. Plexus lesions are observed in very young 
children (obstetrical palsies), spinal lesions in children of any age, and cere- 
bral lesions in children under the age of four chiefly ; but the after-effects of 
these conditions are often visible late in life. From the causes enumerated 
above, as well as in cases of neuritis and progressive muscular diseases, the 
paralysis may be incomplete and restricted to definite groups of muscles only. 
To test the extent of paralysis, the patient should be asked to perform the 
various movements, such as raising the shoulder, putting out arm, flexing 
and extending the elbow, wrist, and fingers. In very young children these 
tests cannot always be satisfactorily made, but even in infants much can be 
inferred from their power to hold or to grasp objects placed in front, above, 
or below them. In older children further tests can be made by offering re- 
sistance to active movements, and asking them to overcome it. The ability 
to bring the fingers in opposition to one another, to make the thumb touch 
the tips of the other fingers, the moving of the fingers to and from the middle 
one, and the ability to write, are necessary tests of the action of the intrin- 
sic muscles of the hand. 

Muscles of Arm, Forearm, and Hand. 









Diseases in 


Name of 


Normal Symptoms of Defi- 


Innervated 


Represented which Muscle is 


Muscle. 


Function. cient Action. 


by 


in 


commonly In- 
volved. 


Triceps. . . 


Extends fore- Arm cannot be ex- 


Musculo- 


Six th, s e v- 


] Poliomyelitis 




arm ; long tended except by 


spiral. 


"enth, eighth 


and other 




head of t r i-j its own weight ; if 




cervical 


affections of 




ceps, and cor- long head of tri- 




segments. 


cervical 




aco-brachialis ceps is affec ted 






cord ; trau- 




help to keep subluxa t i o n of 






matic inju- 




head of hu- 


head oi humerus 






ries ; amyo- 




merus in posi- 


occurs easily. 






\ trophies 




tion. 








j and dystro- 


Biceps 


Flexion and Flexion deficient, 


Musculocu- 


Fourth, fifth, 


phies (tri- 




supination of 


but can be carried 


taneous. 


sixth cervi- 


ceps es- 




forearm. 


out in part by 
other muscles. 




cal. 


capes in 
many pe- 
1 ripheral 
j palsies). 


Supinato r 


Flexes fore- 


Flexion and prona- Musculo- 


Fourth, fifth As "above ; in- 


longus 


arm and aids 


tion deficient; 


spiral. 


cervical, j 


volved in pe- 




in pronation. 


muscle does not 
stand out pro mi- 
ne ntl y if arm is 
flexed and at- 
tempt is made by 
another to extend 
it forcibly; if mus- 
cle is atrophied 
arm is spin d 1 e- 
shaped 






ripheral neu- 
ritis (trauma- 
tic), not in 
lead palsy. 


Supinato r 


S u p i n a t e s Deficient supina- Musculo- 


Fifth cer- 


Diseases as 


brevis. 


han d when tionofhand. 
forearm is ex- 
tended. 


spiral. 


vical. 


above ; also 
in peripheral 
palsies. 



INTRODUCTION— METHODS OF EXAMINATION. 



23 




Fig. 13.— Motor Points on Shoulder and Arm. (Bernhardt.) 



Supinator Longus 
Extensor Rad. Long- 
Extensor Rad. Brev 
Extensor Digitorum Communis 
Extensor Indicis' 

Abductor Pollicis Longu: 
Extensor Pollicis Brevis- 



Dorsal Interosseil. and II. 




Extensor Carpi Ulnaris 
Supinator Brevis 

Extensor Minimi Digiti 
Extensor Indicis 

Extensor Pollicis Longus 



Abductor Minimi Digiti 
Dorsal Interossei III. and IV. 



Fig. 14.— Motor Points of Forearm and Hand. (Erb.) 



24 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Arm, Forearm, and Hand. — Continued. 



Name of 
Muscle. 



Exten s o r 
carpi 
rad i a 1 i s 
longus et 
brevis. 



Exten- 
sor carp i 
ulnaris. 



Exte nsor 
d i g i t o- 
rum com- 
munis. 

Extensor 
indicis. 

Ext en s o r 
minimi 
digiti. 

F 1 e x o r 
c a r p i| 
radialis. | 

Fie x o r 
c a r p 
ulnaris. 

Pal m a r is 
longus. 



F 1 e x o r 
di gi to- 
rum sub- 
limis. 

F 1 e x o r 
d igi to- 
rum pr o- 
fundus. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Diseases in 
T , „ , which Muscle is 

Innervated Represented commonly In . 

b y in volved. 



Extens ion Wrist cannot be'M use u 1 o- Seventh cer- As before ; es- 

vical. peciallyin 

neuritis. 



and abduc- 
tion of wrist ; 
the shorter 
muse 1 e h a s 
pure ex ten- 
sion action 
only. 
Extension and! Wrist 
abduction ot 
wrist. 



flexed dor sally spiral, 
(extended) or ab- 
ducted ; flattening 
of forearm. (Fig. 
ii.) 



As above. 



canno t be 

flexed dorsally or 

adducted ; " drop 

I wrist " is charac- 

I teristic of paraly- 

j sis of extensors. | 

] j B'irst pha langes Musculo 

| cannot be ex- spiral. 

Extension of j tended nor fingers 

I first pha-| abducted ; grasp 

\ langes of all is weak because 

| fingers and flexor muscles are 

abduction. ; sho rtened and 

I cannot contr act 

J I forcibly. 

Flexion o f Deficient flexion. Median. 

wrist and pro-l 

nation. 

Flexion of Flexion and supi- Ulnar, 
wrist a n d| nation impaired, 
supination. 

Flexion o f Flexion impaired ; Median, 
wrist only. no an o m a 1 o u s 

position of hand 
from paralysis of 
wrist as hand falls 
by its own weight; 
the flexors of 
I fingers may act as 
substitutes. 
Flexes second Second ph al an x Median, 
phal a n x to-i cannot be flexed, 
ward first. 

Flexes last Last two phalanges Ulnar and 
two phalanges cannot be flexed. Median, 
toward first. 



Seventh cer- As above, 
vical. 



Seventh cer- As above, 
vical. 



Eighth cer- As above, 
vical. 

Eighth cer- As above, 
vical. 

I 

Eighth cer- As above, 
vical. 



Eighth cer- As above, 
vical. 



Interossei Abduction and 
and lum-j adduction of 
bricales. j fingers if first 
i phalanges are 
i extended; 
flexion of first 
ph a 1 a n g e s 
and siraul- 
tane o u s ex- 
tension of sec- 
ond and third 
phalanges. 



Fingers cannot be 
abducte d or ad- 
duc t e d ; inte r- 
osseous sp a c e s 
are very marked ; 
" Main en griff e " 
due to extension 
of first phalanges 
and flexion of 
second and third 
phalanges. (Figs. 
17- 18.) 



Ulnar, which 
also sup- 
plies th i r d 
and fourth 
lu m b rica- 
les; median 
supplies 
first two and 
sometimes 
third 1 u m- 
bricales. 



Eighth 
vical. 



Eighth 
vical, 
dorsal. 



cer- As above; 
muscle should 
be tested with 
I special care 
in ca s e s of 
traumatic in- 
juries, 
cer- As above ; of- 
fir s tj ten the first 
muscles to be 
affected in 
progre s s i v e 
spinal atro- 
phies. 



INTRODUCTION— METHODS OF EXAMINATION. 



25 



Triceps (External Head 

Triceps (Internal Head 
Ulnar Nervt 



Flexor Carpi Ulnar: 
Flexor Profundus Digitorui 

Flexor Subl. Digitor (III & IV 



Flexor Subl. Digit, dnde.x 
and Fifth Finger) ' 



Palmaris Brevi: 

Abductor Digiti Minim; 

Flexor Minimi Digiti 

Opponens Minimi Digit 



Lumbricales 




Musculo -Cutaneous Nerve 

Biceps Muscle 
Brachialis Anticus 



Median Nerve 
jpinator Longus 

Pronator Teres 
Flexor Carpi Radialis 

Flexor Sublimis Digitorum 

Flexor Pollicis Longus 
Median Nerve 

Abductor Pollicis Brevis 
Opponens Pollicis 

Flexor Pollicis Brevis 

Adductor Pollicis 



Fig. 15.— Motor Points on Inner Surface of Arm. (Erb.) 




Fig. 16.— Extreme Atrophy of Thenar Muscles-" Ape Hand." (Duchenne.) 



26 



THE NERVOUS DISEASES OF CHILDREN. 
Muscles of Arm, Forearm, and Hand. — Continued. 













Diseases in 


Name of 


Normal 


Symptoms of Defi- 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


cient action. 


by 


in 


commonly In- 
volved. 


The nar 


Extends first 


Impairment of ex- 


M usculo- 


First dorsal. 


As before; 


muscles : 


phalanx and 


tension and ad- 


spiral. 


more especi- 


Extensor 


abducts meta- 


duction ; flatten- 






ally in amyo- 


pol 1 i c i s 


carpal b one ; 


in g of ball of 






trophies and 


brevis. 


acts with ad- 
ductor polli- 
cis longus. 


thumb. 






neuritis. 


Exte n s o r 


Extends both 


Deficient extension 


M usculo- 


First dorsal. 


As above. 


pol 1 i c i s 


phalanges of 


and ad d uc t ion ; 


spiral. 






longus. 


thumb ; al so 
adduction ot 
meta c a r p al 
bone and 
backward 
movement of 


second phalanx is 
flex e d toward 
first. 










thumb. 








Abductor 


Abduction of 


Deficient abduc- 


Musculo- First dorsal. 


As above. 


po 1 1 i c i s 


metacar p al 


tion of meta- 


spiral. 






longus. 


bone ; aids in 
flexion o t 
hand. 


carpal bo n e ; if 
this muscle and 
extenso r pollicis 
br e v i s are par- 
alyzed adduction 
results. 








Abduct o r 


1 




Musculo- 






pol 1 i c i s 






spiral. 






brevis. 












Opponens 
pollic is 
and outer 


Opposi tion 


No opposition 


Median. First dorsal. 


As above. 


of thumb. 


movement. 






portion of 












the flexor 












brevis. 


J 










Abduct o r 


Flex first pha- 


No flexion; if 


Median and 




As above. 


pol 1 i c i s 


lanx and ex- 


muscles are par- 


ulnar. 






br e v i s ; 


tend seco n d 


alyzed and atro- 








fie xo r 


phalanx (like 


phied, ape hand is 








brevis 


int e r o s s e i, 


formed. (Fig. 16.) 








and ad- 


also have an 










ductor. 


abduction 
and adduc- 
tion action. 










Flexor 


Fie x e s end 


No flexion of end 


Median. 




As above. 


pol 1 i c i s 


phalanx. 


phalanx. 








longus. 













INTRODUCTION— METHODS OF EXAMINATION. 2J 




Fig. 17. — Extreme " Main en Griffe," after Injury to Ulnar Nerve A. Subluxation of 
Hypertrophied Heads of Metacarpal Bones B. (Duchenne.) 




Fig. 18. — Slight Atrophy of Interossei, and Beginning of "Clawed Hand. 
(Duchenne.) 



THE NERVOUS DISEASES OF CHILDREN. 



Muscles of the Pelvic Girdle and Lower Extremities. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Diseases in 
Innervated Represented which Muscle is 
by in commonly In- 

volved. 



Glute al 

muscles. 



Gluteus maxi- 
mus = exten- 
sion of leg at 
hip and slight 
rotation out- 
ward. 



Gluteus med- 
ius et mini- 
mus = abduc- 
tion of leg ; 
if leg is ex- 
tended also 
lateral move- 

| ment of trunk. 

Outward rota- 

( tion of thigh. 



External 
rotators : 

Pyrifo r- 
mis. 

Gemelli. 

Quadratus 
femoris. 

Internal 

obturator. 

Extern al 

obturator. 



Ilio-psoas. Flexion and 

outward rota- 

| tion of thigh, i 

Tensor Flexion and in- 
fasciae la-j ward rotation, 
toe. 

Adductor 1 Adduction of 
muscles, thigh. 



Inferior Glu- 
teal (sacral 
plexus). 



No extension of 
thigh ; great dif- 
ficulty in climb- 
ing; no abduction 
of thigh; waddling 
gait, exaggerated Gluteal su- 
movement of pel-[ perior. 
vis. 



Deficient outward 1 
rotation; leg | 
turned inward 



Fi r s t a n d In progressive 
second sa- atr o p h i e s ; 
cral seg- these muscles 
ments. are hypertro- 

phied or atro- 
phied in vari- 
ous forms of 
dystr o phy ; 
myelitis of 
lower cord; 
poliomyelitis ; 
traumatic in- 
juries to cord 
! and cauda 
equina. 



Fifth 
bar. 



lum- As above. 



| S a c r a 1 

; plexus 

(muscular 

branches). 



Obtura to r 
nerve (lum- 
bar plexus) 

Crural (lum- Fourth lum- 



Sartorius. Flexion of hip 

and knee.j 

! and slight! 

i outward rota-i 

tion of thigh. | 

Q u a d r i - Extensor of leg 

ceps fe- (rectus femor-! 

moris. is also flexes' 

, hip). 



"1 Flexion diffi- 

cult; in bed bar plexus), bar. 
[ thigh cannot be 
flexed; diffi- 
culty risingSuperior As above, 
from horizontal gluteal. 
position. 
Mo addu c tion; Obtu r a to r Third lum- 
thigh rolls out- nerve, great bar. 
ward. j sciatic and 
crural. 
Flexion impaired ; Crural. Third lum- As above, 
acts imperfectly. bar. 



Leg cannot be ex- Crural, 
tended ; to test it 
ask patient, who is 
lying down with 
hip bent, to stretch 
out the leg ; when 
patient is sitting 
down to extend 
leg ; or try to flex 
leg while patient 
extends forcibly in 
the sitting posture 
with leg hanging 
down. 



Fourth lum- As above 



bar. 



very 



fre q u e n t in 
poliomyelitis. 



INTRODUCTION— METHODS OF EXAMINATION. 29 



Sciatic Nerve 



Biceps Femoris (Long Head 
Biceps Femoris (Short Head 



Peroneal Nerve 



Gastrocnemius (Outer Head 



Flexor Hallucis Longus— 




Fig. 19. 



Crural Nerve 

Obturator Nerve 

Pectinus 

Adductor Magnus 

Adductor Long. 

Crural Nerve 
Vasti Interni 



Gluteus Maximus 



Adductor Magnus 

Semitendinosus 

Semimembranosus 



Tibial Nerve 



Gastrocnemius (Inner Head) 
Soleus 



Flexor Digit. Comm. Longus 
Tibial Nerve 




Tensor Fasciae latae 



Quadriceps 



Rectus Femoris 



Vastus Externus 



Fig. 20. 



$0 THE NERVOUS DISEASES OF CHILDREN. 

Muscles of the Pelvic Girdle and Lower Extremities.— Continued. 



Name of 
Muscle. 



Normal 
Function. 



Symptoms of Defi- 
cient Action. 



Innervated 
by 



Diseases in 
Represented ! which Muscle is 
in commonly In- 

volved. 



Biceps; Flexors of leg, Deficient flexion ; Sciatic. 
Semite n- and extensors action of quadri-j 
di'nosus ofhipinordi- ceps may cause 
and semi- nary walking, excessive ext e n • 
membra-l not in climb-i sion; in standing ; 
nosus. i ing stairs. I thigh is flexed to 
I excess; trunkl 
moved backward. | 
Gastrocne- Flexion and Deficient flexion of Internal pop- 



mms (al- adduction of 
so plan- foot ; toes 
tariusand point inward; 
soleus). first phalang- 
es are extend- 
ed; last pha- 
1 an g e s in 
plantar flex- 
ion. 
Peron e u s Slight flexion, 
longus. chiefly abduc- 
tion of foot ; 
elevates outer 
margin of 
foot. 



foot ; heel cannot liteal. 
be raised ; cannot! 
stand on tiptoes. 



Fifth lum- 
bar. 



Fifth lum- 
bar. 



As before. 



An t e r i o r Extensors 



Deficient ab d u c- Peroneal, 
tion ; plantar arch 
lessened; in- 
creased by con- 
tracture. (Figs. 

I 21-24) J flat-foot ; 

I walking tiresome, 
of Deficient e x t e n - Anterior tib- 



ial. 



tibial foot and toes; 
muscles' tibialis anti- 
(tibialis 1 cus is also ad- 
ant icus,{ ductor; ex- 
extensor tensor digito- 
digito-rum in ad- 
rum, and ! dition to mov- 
exten s o r ing toes, also 
pollicis elevates outer 
longus). ridge of foot 
and abducts I 
foot. 
Poster i o r Adduction o f 1 
tibial foo t ; outer; 
muscle. margin of. J 
foot becomes \ 
J convex. 
Peron eu s Abduction of | 
brevis. foot. | j 

Inteross ei Abduction and Abduction and ad- Poster ior 
pedis et adduction of duction of toes; tibial. 
lumbri- toes; flexors] deficient; paral- 
cales. of first pha- 1 ysis of interossei ; 

lanx ; extend hyperextension of 
second and first phalanges ; 
third phalanx. : second and third 
flexed (clawed 
foot). (Fig. 25.) 
Adductor ; Flex first pha- Deficient flexion of P o s t e r i o r 
flexor lanx of big toe toes; foot cannotj tibial 
brevis and extend be pushed off 
and ab- second. | ground easily 

ductor 
hallucis. 1 



sion; "drop-foot, 
toes scrape floor 
to clear this, ex- 
cessive flexion at 
knee and hip ; 
contr a c t u r e of 
flexors and pes 
equinus or equi- 
novarus. (Figs, 
11, 26, 27.) 



Deficient abduc- Posterior 
tion or adduc- tibial nerve 
tion; deformities 
result from de-| 
ficiencies. j Peroneal. 



First and 

second sa- 
cral. 



Fifth lum- 
bar, first sa- 
cral. 



Most promi- 
nently affect- 
ed in dystro- 
phies and in 
poliomyelitis. 



As above, but 
particularly in 
poliomyelitis ; 
peripheral 
neuritis ; fre- 
quent also in 
peroneal form 
of progressive 
muscular 
atrophy. 



First and 
second sa 
cral. 



First and 
second sa- 
cral. 



As above. 



INTRODUCTION — METHODS OF EXAMINATION. 



3i 




Fig. 21. — Submetatarsal Prominence. A 
Lower than Normal, and Increased 
Plantar Arch, resulting from Contract- 
ure of Peroneus Longus. 



Fig. 22. — Exhibiting, in Addition, Promi- 
nence of the Tendon of the Peroneus 
Longus F, (Duchenne.) 





Fig. 23. — Plantar Surface 
of Same Foot, exhibit- 
ing Changes due to Con- 
tracture of Peroneus 
Longus, shortening of 
Transverse Diameter A 
C, and Torsion of Foot. 



Fig. 24. — Same Foot. 
Valgus Position due to 
Same Cause. 



Fig. 25. — " Clawed Foot " from 
Atrophy of Interossei and Other 

Intrinsic Muscles of Foot. (Du- 
chenne.) 



32 



THE NERVOUS DISEASES OF CHILDREN. 




Fig. 26. — Pes Equinus in a Boy- 
Five Years of Age from Atrophy 
of Tibialis Anticus. 




Fig. 27. — Pes Equinus of 
Eight Years Duration. 



Tibialis Anticus 
Extensor Digit. Commun. Longus. 



Peroneus Brevis 



Extensor Hallucis Longus 



Dorsal Interossei 




Peroneal Nerve 



Gastrocnemius (Outer Head). 
Peroneus Longus 



Flexor Hallucis Longus 



•*- — - — 4- Extensor Dig-it. Comm. Brev. 



>l===&i 



Abductor Minimi Digiti 



Fig. 28.— Motor Points. (Erb.) 



INTRODUCTION— METHODS OF EXAMINATION. 33 

Muscles of Back and Trunk. 





. 








Diseases in 


Name of 


Normal 


Symptoms of 


Innervated 


Represented 


which Muscle is 


Muscle. 


Function. 


Deficient Action. 


by 


in 


commonly In- 
volved. 


Erector 


Extens i n of 


Lordosis of lower 


Dorsal 


S e c n d to 


In spinal dis- 


spinee: 


lower dorsal 


spine ; perpendic- 


nerves. 


12th dorsal 


eases and 


s a c r - 


and lumbar 


ular line from 




segments. 


progr e s s i ve 


lumbal- 


vertebras. 


shoulder falls be- 






muscular at- 


is ; 1 n- 




ll i n d os sacrum; 






rophies. 


gissimus 




unilateral palsy 








dorsi. 




causes deflection 
of spine toward 
sound side. (Fig. 
30.) 








Quadratus 


Deflects lower 


Lateral movements Lumbar 




As above. 


lumbo- 


p r ti n of 


of lower vertebrae 


nerves. 






rum. 


spine lateral- 

iy. 

Aid in expira- 


imperfect. 








Abdomin- 


Lordosis, with pro- 


Dorsal 


S ec n d to 


As above. 


al mus- 


tion ; also in 


trusion of nates 


nerves. 


12th dorsal 




cles. 


voiding blad- 
der and strain- 
ing at stool : 
in k e e p i n g 
vertebras i n 
position. 


and abdomen; 
other actions defi- 
cient ; cannot 
straighten up 
f r m recumbent 
position without 
assistance of 
hands. 




segments. 





Serratus Magnus 



Obliquus Abdominis 
Externus 



Transversus Abdom 
inis 




Rect. Abdominis 



Fig. 29. — Motor Points of Trunk, (Bernhardt.) 



34 



THE NERVOUS DISEASES OF CHILDREN. 



Paralysis of individual muscles is not as frequent in the lower extremities 
as in the upper ; large groups of muscles (the anterior tibial, the posterior 
tibial) are often paralyzed together. Symmetrical palsies are common (in- 
volving thigh groups, both post-tibial groups, etc.). Complete paraplegia of 

the lower extremities is seen in 
some spinal and cerebral lesions of 
children (spastic birth palsies, dor- 
sal myelitis (poliomyelitis and my- 
elitis), and in multiple neuritis. Pa- 
ralysis may be flaccid (spinal or 
peripheral) or spastic (cerebral, or 
spinal). If spinal and spastic, lesion 
is in lateral column. 

To test the exact extent of pa- 
ralysis, the child is to be placed on 
its back ; if it does not move the 
limbs at all voluntarily, suspicion 
of very great paralysis or of exces- 
sive pain is a safe one. 

If there is total paralysis, child 
cannot hold the leg if it be raised 
a few inches ; to make further tests, 
see whether child can abduct, 
adduct, flex, or extend the various 
parts. Failure to abduct may be 
due to contracture of adductors ; 
so also incomplete extension of 
knee may be due to contracture of 
flexors of knee. Contractured mus- 
cles must be considered with par- 
ticular care. In very young chil- 
dren, if it is desirable to determine 
whether there is any actual paraly- 
sis, pinch the toe steadily until the 
child draws the leg away, or may 
be expected to do so. 

With older children special tests 
can be made. Ask child to keep 
thigh flexed while you oppose ; to 
test flexors of thigh, rest your hands 
on its knee and ask it to push it away by lifting up thigh ; to test extension of 
thigh ask it to climb on a chair or observe whether it raises hip in walking. 
Place the child on a table and ask it to swing leg forward and backward ; 
to keep leg straight while the physician endeavors to flex it (for extensors of 
leg), or to keep leg flexed while physician attempts to straighten leg (for 
flexors of leg). To test flexors or extensors of foot and toes, it should be 
asked to perform dorsal and plantar flexion with and without resistance. 




Fig. 30. — Patient showing Lordosis of Verte- 
bral Column, clue to Weakness of the Ex- 
tensors (Erector) of the Spine. 



INTRODUCTION— METHODS OF EXAMINATION. 35 

To raise on tiptoes, or to raise toes while keeping heel on ground are good 
tests for the posterior and anterior tibial groups. Paralysis of certain groups 
is attended by contractures of others. Deformities of the feet (pes equinus, 
equino-varus, and valgus) result from paralysis and contractures of the flex- 
ors, extensors, and of the intrinsic muscles of the feet. 

Examination of the gait is of the utmost importance ; 
an attempt should be made to have the child walk ; and 
even if it cannot walk, the position of the legs in stand- 
ing, or in attempting to walk, is often quite characteristic. 
Cross-legged position and cross-legged progression occur 
in children (spastic paraplegia or diplegia) ; the gait may be 
paretic, spastic, ataxic, or a combined form of all three. If 
the gait is simply paretic the child drags its legs carefully, 
walking slowly and without raising the feet. The spastic 
gait is common in the hemiplegias and other cerebral palsies 
of childhood ; also in spinal palsies (myelitis and disease of 
the lateral columns). The knee is stiff, the leg is moved as a 
whole, and the child walks either on the ball of foot or on its 
toes. The ataxic gait, variously modified, occurs in neuritis 
in cerebellar disease, and in hereditary ataxy. In walking 
the child reels, its legs are moved extravagantly, and it 
walks with a broad base. A swinging gait is very frequent 
in poliomyelitis. The gait varies according to the muscles 
affected ; in dystrophies, the gait is apt to be waddling, or 
a mixture of the waddling and swinging gait. 

To be efficient, muscular action must be well directed. The contractions 
of muscles may be entirely normal, but unless the functions of muscles act- 
ing together are properly co-ordinated, the contractions may fail of their prop- 
er effect. In all movements (in walking, standing, writing, grasping, talk- 
ing), co-ordination of the muscles therein concerned is essential. We speak 
of inco-ordination or of ataxia, if movements go wide of the mark. If a per- 
son, instead of touching the end of the nose with the tip of the fingers (.eyes 
being closed), fumbles all over the face, he has ataxia of the upper extremities : 
and if instead of preserving his balance accurately in walking, a person sways 
from one side to the other, and to keep his balance at all walks with a broad 
base, that person has an ataxic gait. The ataxia may result not merely from 
inco-ordination of the muscles, but from insufficient sensory (muscular) im- 
pressions of the whereabout in space of various parts of the body. It a 
person is asked to cross one knee over the other (with eyes closed), he must 
know exactly where the other knee is, if he is to perform the movement with 
nicety; and he must be able to gauge the exact extent of muscular action to 



36 THE NERVOUS DISEASES OF CHILDREN. 

be employed. With the assistance of vision muscular co-ordination is more 
perfect. In children ataxia is less frequent than in adults ; it occurs in mul- 
tiple neuritis, in hereditary ataxy, and in cerebellar disease. (In the last- 
named, there is considerable reeling in addition to mere ataxia ; there is 
inco-ordination and great uncertainty.) 

Before leaving the muscular system it is well to note the tonus of the 
muscles. In children this is of importance ; for by the mere handling of a 
leg the experienced physician will be able to determine whether muscles are 
normal, flabby, atrophied, or contractured. Every normal muscle, if struck 
lightly with a percussion hammer, or with a finger, will exhibit a wavelike con- 
traction. This mechanical excitability may be increased, so that the entire 
muscle contracts forcibly upon the slightest tap (tapping the quadriceps 
femoris may produce movement like that of the knee-jerk) ; or instead of 
single contractions, small fibrillary tremors may be observed on tapping 
atrophied or degenerated muscles. 

Sensory Disturbances are not easily determined in 
children. Many of the finer tests cannot be applied at all, 
for young children are not sufficiently intelligent to give 
reliable answers. The disturbances of sensation may be 
either subjective or objective. 

i. Subjective sensations may vary according to character 
of sensory perception ; thus we may have subjective sensa- 
tions of heat, of cold, of pressure ; if these sensations are in- 
tense, they may be equivalent to pain (Goldscheider) ; there 
may also be perverted subjective sensations, such as formi- 
cations and other paraesthesias (feeling as though a part 
were swollen, enlarged, unusually heavy and the like). Pain 
may vary in character (boring, cutting, thumping, burn- 
ing) ; its description, as Goldscheider has shown, depending 
very largely upon well-known sensory impressions that 
have been associated with pain. Distribution of pain will 
vary ; it may follow the distribution of nerves (neuralgia 
and neuritis); it may be restricted to definite areas, and 
such areas of pain may hold definite relations to disease 
in distant parts (according to Head, Dana, Goldscheider, 
and others). Pain persistently referred to one spot is desig- 
nated as Topoalgia. 

2. In testing for objective disturbances of sensations, 
we must note that ordinary sensation is of a fourfold char- 
acter ; that we have perception of touch, of pain, of heat 
and cold, and of muscular position, and all of these may be 



INTRODUCTION— METHODS OF EXAMINATION. 37 

equally disturbed (complete anaesthesia), or that they may 
be dissociated (partial anaesthesia). 

To test sensation at the bedside, the simpler the tests 
the better; the aesthesiometer can be disregarded altogether. 
To determine tactile sense, passing cotton over the skin is 
generally quite sufficient ; or still better, let the physician 
close his eyes and touch the patient ever so lightly ; his 
own perception of contact will tell whether that of the 
patient is above or below his own ; asking the patient to 
state in which direction a finger is being passed is another 
good test ; a correct answer implies normal tactile percep- 
tion at every point ; writing numbers on the skin may be 
tried in older children. For pain, we may test by prick- 
ing with a pin, by using faradic current, by pinching and 
the like. In all these tests the child's facial expression should 
be studied carefully. To determine the temperature sense 
it is best to employ test-tubes with hot and cold water, or to 
place metallic objects on the skin and see whether differ- 
ences in heat or cold can be made out. Goldscheider has 
shown that there are special areas in the skin for the per- 
ception of pressure, of heat and of cold ; and that the acute- 
ness of sensory perception varies in different areas. 

His test for minimum or maximum heat and cold per- 
ceptions are not practicable, however, at the bedside — least 
of all in children. 

The muscular sense itself is complex. It includes a per- 
ception of passive movements, of active movements ; a per- 
ception of pressure and of resistance ; and a perception of 
the position of parts. 

To test the perception of passive movements it is best to take the joint 
firmly between the two hands ; then move the joint slightly and ask whether 
patient knows what has been done ; a deficiency of this sense can be made out 
easily. This test is an important one, for many cases of ataxia are depend- 
ent upon the loss of this special perception. To test perception of weight, it 
is best to use rubber balls of same size, but filled with varying quantities of 
shot. The balls are placed on the hand, on the leg. etc.. and the patient is 
asked to determine which is lighter or heavier. The patient's knowledge of 
the position of parts is tested by placing one part (a leg. a finger), in a def- 
inite position, or raising it to a definite height (eyes closed) and then asking 
patient to put a symmetrical part in relatively the same position. 

Loss of sensation is termed anaesthesia; diminished sensation hyp.; 



38 THE NERVOUS DISEASES OF CHILDREN. 




merh'anu-s 
Fig. 32. Fig. 33. 

Distribution of the Sensory Nerves. (Freund.) Figs. 31-37- 



INTRODUCTION— METHODS OF EXAMINATION. 39 




40 THE NERVOUS DISEASES OF CHILDREN. 

sia ; and increased sensory perception is termed hyperesthesia. Disturb- 
ances of sensation may vary with the distribution of sensory nerves (Figs. 
6, 31-37). Hemianesthesia implies loss of sensation in one half of the body. 
This is rare in children, and occurs chiefly in hysterical affections. Anoma- 
lies of temperature sense are observed in gliosis of cord and in some cases 
of neuritis. 

Examination of Reflexes. — We distinguish between the deep or 
tendon reflexes, and the superficial or cutaneous reflexes. The former con- 
stitute the more important class. Among the deep reflexes the patella-tendon 
reflex or knee-jerk has the greatest clinical significance ; next in order is the 
ankle clonus, or Achillis-tendon reflex, and in many cases the behavior of 
the wrist reflex, the triceps reflex, and even the jaw-jerk is carefully to be 
considered. In children it is more difficult than in adults to elicit the deep 
reflexes, for the former cannot relax their muscles easily, and thus inhibit to 
a degree every reflex movement. In testing for the knee-jerks and for other 
reflexes, it is best to take the child unawares, before it has learnt to know 
what the test means. While busying one's self with other parts of the body, 
or while talking to the child, if the leg is in a favorable position, strike the 
tendon smartly, and the reflex movement will ensue if the conditions are 
normal. If this first test does not succeed, seat the child at the edge of a 
table with its legs dangling, and then quickly strike the tendon. If the child 
continues to inhibit the reflex, I am in the habit of placing my left hand be- 
tween the crossed knees of the child in such a way as lightly to grasp the 
posterior surface of the upper leg. The child is compelled to relax its 
muscles, and I can easily tell whether it makes the slightest effort to contract 
them. If the tendon is struck, the excursion of the leg is bound to follow, 
provided the reflex is present. In older children Jendrassik's method 
(clinching the fists while the test is made) may be employed ; but it is still 
better for the physician to ask the child to squeeze his fingers the very in- 
stant he strikes the tendon. 

To elicit the ankle clonus, it is best to perform sudden dorsal flexion of 
the foot, while keeping the knee resting on the other hand and in the position 
of moderate flexion. The same clonic movements can be elicited by striking 
the Achillis tendon while the foot is in moderate dorsal flexion. 

The paradoxical muscular contraction described by C. Westphal — a tonic 
contraction of a muscle when suddenly relaxed — is a mere curiosity without 
any known clinical significance. 

The wrist reflex is tested by striking the radial side of the forearm near 
the wrist ; a smart tap will cause contraction of the supinator and biceps 
muscles. The triceps reflex is elicited by striking the tendon while the arm 
is flexed. The " jaw jerk," a reflex contraction of the masseter, is brought 
about by striking the middle of the chin when the mouth is slightly open, or 
by tapping a pencil laid on the lower teeth or jaw. I have seen this distinct- 
ly present in a case of multiple sclerosis in a child. 

The power of inhibition is not so great over the other 
reflexes as over the knee-jerks ; and in testing for the wrist 



INTRODUCTION— METHODS OF EXAMINATION 4 1 

or triceps reflexes, the most important point is to place the 
joints in a position of moderate flexion so as to give the 
muscles the fairest chance for contraction. The deep re- 
flexes may be present, diminished or exaggerated. The 
knee-jerk is present under ordinary conditions ; the mere 
presence of the ankle clonus is abnormal ; and the reflexes 
in the upper extremity are present in some and absent in 
other normal individuals. 

The reflexes are diminished or absent in peripheral 
nerve disease, in spinal-cord affections involving the pos- 
terior columns and the gray matter of the cord ; in disease 
of the cerebellum. They are exaggerated in diseases of the 
brain and in diseases involving the lateral columns of the 
cord ; also in some functional diseases. 

The diminution of the reflex is determined easily enough ; 
but the absence of the reflex is more significant than a mere 
diminution. The diminution or absence of a reflex may be 
apparent only. Thus the knee-jerk may be wanting in con- 
sequence of contraction of the posterior flexors of the leg, 
or there may be anchylosis of the joint preventing free 
movement ; but in such cases the contraction of the quadri- 
ceps can be seen or felt upon tapping the tendon. In some 
children the tendon is displaced or so imbedded in fat that 
it cannot be struck readily. 

It is more difficult to state when a reflex is exaggerated. 
In this matter comparison based upon experience is the 
best guide. We can safely speak of an exaggerated re- 
flex if there is an exceeding quickness of the response, if 
the slightest tapping produces a liberal movement ; or if 
clonic movement results from a single blow (as in patella 
clonus). If the reflex can be elicited indirectly by striking 
the finger placed over the tendon, the reflex is surely exag- 
gerated. Moreover, if a child of three years or more 
is so seated that its feet are in solid contact with the floor, 
and if when its patella tendon is struck, the foot is lifted 
from the floor, the reflex is considerably increased. 

Very lively knee-jerks do not necessarily imply organic 
disease, but exaggerated knee-jerks with ankle clonus are 
rarely due to functional disease. Exaggeration or absence 
of the reflexes in one half of the body is always suspicious 



42 THE NERVOUS DISEASES OF CHILDREN. 

of organic disease, and so is the association of increased 
mechanical excitability and of contractures with increase 
of the reflexes. 

The superficial or cutaneous reflexes are not of great importance in child- 
hood. The abdominal, epigastric, and cremasteric reflexes are but poorly 
developed in children ; the last named is absent in many older children ; and 
no significance can be attached to its presence or absence unless the con- 
dition is not the same in both halves of the body. The plantar reflex (re- 
traction of the foot on irritation of the soles) deserves a few words of 
comment. Like the other cutaneous reflexes it is absent in diseases of the 
peripheral nerves, and in diseases of the spinal cord involving the course of 
the reflex arc ; if the lesion is higher than the level through which the arc 
passes, the reflex may be exaggerated, but this is not as constant a feature 
as is the case with the tendon reflexes. In all unilateral brain-lesions the 
cutaneous reflexes are absent as a rule on the paralyzed side. The plantar 
reflex is absent in condition of unconsciousness (as w r ell as in deep sleep), 
and its presence or absence may give a clew as to the degree of coma in a 
given case. 

ELECTRICAL EXAMINATION. 

The distribution of a paralysis, the flaccid or spastic con- 
dition of the muscles, the behavior of the reflexes, may 
enable us to make an accurate diagnosis in many cases, but 
in many more the diagnosis cannot, be safely established 
until a careful electrical examination has been made.* 

The chief object of such an examination is to determine 
the response of various muscles and nerves to the faradic 
and galvanic currents, and to compare such response with 
the conditions obtaining during health. 

The younger Westphal has shown that the nerves and 

* The author cannot undertake to give a detailed account of medical elec- 
tricity ; for this the student is referred to the treatises of de Watteville, of Beard and 
Rockwell, of Erb, Bernhardt or Lewandowski ; also to the special chapters in the text- 
books of Gray, Dana, and Gowers. The importance of a thorough knowledge of elec- 
tricity should not be overlooked. Whatever one may think of the value of electricity 
as a therapeutic agent, there can be no question of its great merit as an aid to diag- 
nosis. It is disappointing to find so good an observer as Moebius disputing the value 
of electricity in diagnosis. (Neurologische Beitrage, i., pp. 97-100.) Moebius argues 
that the experienced neurologist can make a diagnosis without the assistance of elec- 
trical tests, and that such tests are not wholly reliable. But they are reliable in fully 
ninety-five per cent, of all cases, and as for making a diagnosis without the aid of elec- 
tricity, that may be possible. A clever neurologist can also diagnosticate a cere- 
bral tumor often enough without the ophthalmoscope, yet he will not spurn the cor- 
roborative evidence which an examination of the papillae may furnish. 



INTRODUCTION— METHODS OF EXAMINATION. 43 

muscles of the new-born, and of children up to the age of 
live weeks, do not respond except to very strong currents, 
and even then the contractions are slow. But after that age 
the normal nerve and muscle will respond to faradic and 
galvanic stimulation. A knowledge of the position of the 
motor points is essential. (See Figs. 7, 13, 14, 15, 19, 20, 28, 
29.) 

Always begin the examination with the faradic current, and apply the 
one pole to the nerve or muscle to be examined, and place the other pole at 
a safe distance, say on the sternum. Use mild currents (children grow very 
restless under strong currents), and determine the weakest current with which 
a contraction is to be obtained, or whether any contraction can be obtained 
at all, or not. Place the arm or leg in a position favorable for contraction 
of the muscles to be tested, and make sure that the contraction which en- 
sues is the one that should naturally follow. Do not, for instance, claim that 
the electrical conditions are normal if on applying an electrode to the ex- 
tensor digitorum communis, a flexion contraction of wrist and fingers ensues. 
And if there is any doubt as to the strength of the current, let the physician 
apply it to himself ; he will be more certain to be merciful if he does ; or if 
there is a doubt whether a nerve or muscle should respond to a given strength 
of current, let that same current be applied to the same point in the opposite 
half of the body. Thus we can compare two peronei with each other ; or we 
can compare a peroneal nerve with a facial or a median nerve of the same 
or opposite side of the body,* or we can compare the degree of excitability 
with the figures given in tabular form by Stintzing for the minimum and 
maximum currents required to produce contractions of various nerves and 
muscles.! 

A simple diminution of faradic and galvanic response of nerves and mus- 
cle occurs in light cases of peripheral neuritis, in hysterical conditions, in 
atrophies following joint disease or disuse, and in many of the primary dys- 
trophies. A simple and continued increase of response is observed practi- 
cally only in cases of tetany. But in addition to mere quantitative changes 
there are also changes in the character and quality of the response to be ob- 
served in cases of degenerative disease of the peripheral nerves, of the mus- 
cles, and of parts of the spinal cord. The changes implying degenerative 
disease are referred to as the reaction of degeneration. 

* In children the motor points may be obscured by deposits of fat. 

t American faradic batteries are now provided with a sliding scale which enables the 
physician to record how far apart the primary and secondary coils wore when first con- 
traction was obtained. In recording the effect of the galvanic current, the strength 
should be stated in milliamperes. 



44 THE NERVOUS DISEASES OF CHILDREN. 

The following table will give the salient features of nor- 
mal electrical conditions and of the reaction of degenera- 
tion : 



Normal Electrical Coxditioxs. 



Reactiox of Degexeratiox. 



Nerve and Muscle. 



Nerve. 



Muscle. 



Faradic cur- Contractions good 
rent. prompt. 



No response No response (except in partial 
(except in R. D.). 
partial R. j 
D.L 



Galvanic Contractions prompt and No response, 
current. quick. 



Order of i. K. C C* 

contrac- 2. A. C C. / interchange- 



3. A. O. C \ able. 

4. K. O. C. 

5. K. C Te. (rare). 



Increased excitablity at first, 
then diminished ; contrac- 
tions sluggish. 



A.C.OK.C.C. 



With stronger currents. 

1. A. C C. 

2. K. C. C 
3 A. O. C 
4. K. O. C 

or 
A. C. C. = K. C C. 



* K. C C = Kathodal closure contraction ; A. C C = Anodal closure contrac- 
tion ; A. O. C = anodal opening contraction ; K. O. C. = Kathodal opening con- 
traction ; K. C Te. = Kathodal closure Tetanus. 

The preceding table states that in the reaction of degeneration (R. D.) 
the nerves and muscles fail to respond to the faradic current, but that the 
muscles continue to respond to the galvanic current in altered fashion ; first 
of all, the contractions are sluggish, and this is the most important point, and 
secondly, the order of contractions is reversed ; the anodal closure contrac- 
tion can be obtained with weaker currents than are required for the kathodal 
closure contraction. 

The mistake is commonly made to suppose that there is 
no reaction of degeneration present unless the galvanic order 
of contractions is reversed ; this occurs in the majority of 
typical cases, to be sure ; but the failure of response to the 
faradic current is early evidence of a reaction of degener- 
ation, and often precedes the development of abnormal gal- 
vanic conditions. 

In first examinations the faradic test is therefore much 
the more important. 

Many recent authors (including Remak) agree further- 
more in considering the sluggishness of contraction much 
more significant of degenerative changes than the reversal 
of the normal formula. 



INTRODUCTION— METHODS OF EXAMINATION. 



45 



It should be remembered also that muscles may be so 
much atrophied as to give no contraction to the strongest 
galvanic current. 

A partial reaction of degeneration is often overlooked ; it implies a mere 
diminution of faradic excitability of nerve and muscles, but the response of 
the muscle on direct galvanic excitation is slow, and the formula may be re- 
versed. Even if the faradic response is present, it is important to make sure 
that the galvanic response is prompt, not sluggish. 

The greatest service which electricity affords us in diag- 
nosis is in differentiating between cerebral diseases on the 
one hand and certain spinal and peripheral diseases on the 
other. The ganglion cell of the anterior horn and its ana- 
logue in the cranial nerve nuclei are responsible for the nor- 
mal electrical condition of peripheral nerves and muscles. 
Lesions involving this cell, or interfering with the transmis- 
sion of impulses from it to the periphery are attended by 
changes in electrical reactions; in all other diseases the 
electrical conditions are practically unaltered. 

Whence it follows that the electrical conditions are : 



Normal in 



i. All cerebral diseases,* excepting those 
of cranial nerve nuclei. 

2. Diseases of lateral and posterior col- 

umns of spinal cord. 

3. Functional troubles. 

4. Mild peripheral troubles. 

5. In some forms of muscular dystrophy. 



Distinctly Altered (R. D.) in 



1. Bulbar paralysis (acute and chronic). 

2. Polioencephalitis superior (Wernicke). 

3. Poliomyelitis (acute and chronic). 

4. Progressive amyotrophies. 

5. Amyotrophic lateral sclerosis. 

6. Myelitis, but only in muscles repre- 

sented in diseased level. 

7. Gliosis and tumor involving gray mat- 

ter. 

8. Anterior root disease (syphilis, tumor, 

etc.) 

9. Vertebral disease (Pott's disease, tu- 

mor). 

10. Peripheral neuritis (traumatic, rheu- 

matic, toxic). 

11. In some forms of muscular dystrophy. 



A very few exceptions have been reported. 



To complete the examination, be sure to look for 
trophic symptoms which occur in many functional disor- 
ders as well as in disease of the peripheral nerves and in 
some diseases of the spinal cord (gliosis, tumor); and finally 
the conditions of the vesical and rectal reflexes should be 



46 THE NERVOUS DISEASES OF CHILDREN. 

recorded ; though in regard to them the influence or lack 
of previous training of the child must be taken into ac- 
count. A sudden loss of control is often associated with 
serious cerebral and spinal disease. , 

BIBLIOGRAPHY.* 

Dana : Text-book of Nervous Diseases, 1893. 

Duchenne : Physiologie des Mouvements. Paris, 1866. German translation 

by C. Wernicke, Berlin, 1885. 
Fisher and Peterson : Cranial Measurements in Twenty Cases of Infantile 

Cerebral Hemiplegia. New York Medical Journal, April 6, 1889. 
Goldscheider : Diagnostik der Nervenkrankheiten, Berlin, 1893, and Ueber 

den Smerz, Berlin, 1894. 
Gowers: Diseases of the Nervous System. Second American edition. 1892. 
Gray : Nervous and Mental Diseases, 1893. 
Moebius : Diagnostik der Nervenkrankheiten, Leipzig. Second edition. 

1894. Neurologische Beitrage, Heft I., 1894. 
Oppenheim, H. : Lehrbuch der Nervenkrankheiten. Berlin, 1894. 
Oppenheimer, Z. : Schmerz und Temperatur Empfindung, Berlin, 1893. 

(On Pain and Temperature Sense.) 
Remak : Deutsche Medicinische Wochenschrift, November 16, 1893. 
Preyer : Die Seele des Kindes. Third edition. Berlin, 1890. 
Sternberg: Die Sehnenreflexe. Leipzig, 1893. 
Stintzing : Deutsches Archiv f. klin. Medicin, vol. xxxix., p. 76. 
Westphal, A. : Archiv fiir Psychiatrie, vol. xxvi., p. i., 1894. 

* This list is necessarily incomplete ; it is intended to give the more recent works 
and articles chiefly which have been especially consulted in the preparation of this 
chapter. 



PART I. 



GENERAL NERVOUS DISEASES. 



CHAPTER II. 

CONVULSIONS— ECLAMPSIA INFANTUM. 

The peculiar seizures so common in ii fancy, and desig- 
nated as convulsions, constitute a symptom, not a form, of 
disease. The unusual frequency of convulsions in early 
childhood points to the greater excitability in the child of 
the motor mechanism of the brain. The motor centres in 
the cortex are more apt to " discharge," and the inhibitory 
power of the brain is less developed than in the adult. 
Eclampsia has been found to be a convenient term for the 
condition in which convulsions are apt to occur, but it 
shoald be remembered that it is neither a disease nor a 
sufficient diagnosis in any given case, for eclampsia in 
children, as well as in adults, may be the result of many 
widely different disorders. 

However frequent these convulsive seizures may be in 
early life, healthy children are as exempt from them as are 
healthy adults. If they are not the result of organic disease 
of the brain, their occurrence indicates functional involve- 
ment of the brain in connection with disease in other parts 
of the body. 

A convulsion is a motor discharge resulting in muscular 
contractions of one or more parts of the body. In the major- 
ity of cases these convulsive movements are associated with 
a number of other symptoms ; viz., an initial cry, turning 
of the eyes upward or inward, very sudden loss of conscious- 
ness, involuntary passage of urine and faeces, prolonged 
drowsiness, and a condition of stupor if not of coma. The 
muscular movements may be tonic at first, but are apt to 
lead to clonic spasms. No two convulsive seizures are 
exactly alike, but the student who has seen a single seizure 
will not forget the main features of the condition. The 
4 



50 THE NERVOUS DISEASES OF CHILDREN. 

convulsion may come on without warning, or the child may 
have complained of uncomfortable sensations in the head or 
stomach, of a little dizziness or nausea; the child gives a 
shrill cry and at once is thrown into tonic and clonic con- 
vulsions. These may be partial or general, unilateral or 
bilateral. The legs are stiff, the arms bend ^it the elbow, 
the wrists are flexed, and the hands clenched, with the 
fingers firmly closed upon the thumb. The head is thrown 
back or rotated to one side ; the back may be arched, the 
face is pale at first, but soon reddens and the eyes remain 
wide open. The pupils do not react. In older children 
the tongue may be caught between the teeth, and bloody 




Fig. 38. — Child of One Year Photographed During a Severe Convulsive Seizure. 

froth may be noticeable at the lips. In very young chil- 
dren Ave have the ordinary foam at the mouth. The respi- 
ratory muscles are in a condition of spasm ; the child often 
turns blue rapidly and is in danger of asphyxia. Fortu- 
nately the tonic spasm soon ceases, slighter clonic convul- 
sions then set in, and after a little the child groAvs quiet, 
and is apt to be in a dazed or stuporous condition for a 
period that may vary from several minutes to as many 
hours or days. While the clonic spasm lasts there is im- 
minent danger of injury to the child's head and limbs in 
consequence of the severe jactations, but this danger is not 
as serious as that from asphyxia during the period of tonic 
spasm. 

Causes. — Convulsions always denote " cerebral (cortical) 
irritation." It might be sufficient to state that any morbid 



CONVULSIONS— ECLAMPSIA INFANTUM. 5 1 

process which brings about cortical irritation, directly or 
indirectly, is liable to cause convulsions. But it will be 
better to enumerate the chief conditions under which in- 
fantile convulsions occur. 

I. Convulsions occurring within the first few days of 
life are, as a rule, the result of meningeal hemorrhage, due 
either to protracted labor, or to instrumental delivery. If 
the child survives, the injury done to the brain often leads 
to the development of spastic palsies. (Cf. chapter on Cer- 
ebral Palsies.) 

Ii. The convulsions may be due to organic disease of the 
brain, such as tumor, abscess, meningitis, or vascular lesions ; 
in all of these cases they may be the earliest symptom of 
the disease, but other symptoms associated with them, such 
as headache, paralysis, optic neuritis, and the like, will be 
forthcoming. 

III. They may mark the onset of any acute infectious 
disease. The initial convulsion is almost as frequent in 
children as is the initial rigor in the adult. The former is 
the infantile equivalent of the latter. It is extremely com- 
mon at the onset of pneumonia, scarlet fever, and measles ; 
and I have also witnessed convulsions in malarial fevers, and 
at the onset of chicken-pox. The general practitioner is 
very apt to err in the interpretation of such convulsions. 
A child that has had a convulsion, preceded or followed by 
a rise of temperature, is often suspected to be in the first 
stages of a meningitis or of some other cerebral disease. 
While this is true in some instances, the fact is entirely 
overlooked that the seizure may be the initial symptom not 
only of any of the ordinary infectious diseases, but of the 
acute spinal diseases as well — poliomyelitis, for instance 
(probably an infectious disease). 

IV. The convulsions may be of reflex origin. Almost 
every possible peripheral disturbance has been supposed bv 
one author or the other to be a sufficient cause of convul- 
sions. I need merely mention ocular insufficiencies, nasal 
obstruction, narrow prepuces, not to call up a dozen or 
more about which there has been much, and often bitter, 
discussion. Of the influence of two conditions there can be 
no reasonable doubt. The first of these is dentition ; the 



52 THE NERVOUS DISEASES OF CHILDREN. 

second, gastrointestinal irritation. There has been some 
question whether a tooth about to cut through the gum can 
cause an eclamptic seizure. Delayed dentition is so fre- 
quently associated with rickets that the latter condition is 
considered by many to be the chief factor, and this I be- 
lieve to be true ; but convulsions do at times occur in chil- 
dren who present no tangible signs of rickets. Moreover, 
there is good reason why a cutting tooth should produce 
convulsions in a child so disposed, say by rickets, or any 
form of exhausting disease. " The cutting tooth " is a direct 
irritant to the filaments of the trigeminal nerve, which car- 
ries this irritation easily enough to the convulsive centres 
at the base of the brain. I have little doubt of this special 
reflex origin of convulsive seizures since observing in the 
adult the occurrence of severe epileptic paroxysms in a case 
of trigeminal neuralgia. 

The influence of gastro-intestinal irritation is well illus- 
trated by the convulsions occurring in the course of an 
acute or chronic intestinal catarrh, in the ordinary summer 
diarrhoea of young children, or with the exhausting chronic 
diarrhoea in older children. The same phenomena are apt 
to occur in the presence of animal parasites (pin-worms, 
and, above all, tape-worms), and I have known severe con- 
vulsions to disappear promptly after the removal of a tape- 
worm in children between the ages of four and eight years 
never to return again. 

V. Convulsions may be due to poisons (organic or me- 
tallic) circulating in the blood. Under this heading we 
may include the convulsions of uraemia, those due to the 
presence of ptomaines in the stomach and bowels, and con- 
vulsions produced by the administration of lead and other 
metallic poisons. Toxsemic convulsions are not as common 
in the child as in the adult, unless, following recent studies 
by Chenbach and others, we consider the convulsions of in- 
fectious fevers and of intestinal troubles to be due to some 
form of auto-intoxication. 

VI. Convulsions may result from severe loss of blood, 
from any exhausting disease, or from such constitutional 
disturbances as scrofula and rickets. A vast literature has 
grown up on the subject of rickets and convulsions. What- 



CONVULSIONS— ECLAMPSIA INFANTUM. 53 

ever theory one may be willing to adopt in order to ex- 
plain this close relationship, the fact remains that an enor- 
mous percentage of children having convulsions are af- 
fected with rickets. 

An hyperasmic condition of the cortex analogous to the 
condition of the bones in rickets, is the only explanation we 
can give of the frequency of convulsions during rickets, 
unless we are satisfied to accept the very vague statement 
that the latter is a simple predisposing cause of convul- 
sions. 

VII. A convulsion may be idiopathic, hereditary, or, if 
you choose, the first incident in the course of an ordinary 
epilepsy. This possibility must be kept in mind, but it is a 
more probable explanation if the child happens to be above, 
rather than under, four or six years of age. This inter- 
pretation of a convulsive seizure occurring in a child should 
be given only after excluding every other possible cause. 

It is surprising, but a fact nevertheless, to which I have 
called attention a number of times, that the more carefully 
we scrutinize cases of convulsions, or of epilepsy for that 
matter, the fewer of them appear to be truly idiopathic. It 
will be well not to make the diagnosis of an incipient epi- 
lepsy unless we are informed that the child has had previous 
convulsions at somewhat long intervals, or unless we have 
occasion to observe subsequent convulsions without a dis- 
tinct additional cause for each seizure. 

VIII. Convulsions occur after traumatic injury. In some 
cases actual hemorrhage over the motor centres is the im- 
mediate cause of the convulsions. In other cases no tan- 
gible injury to the brain has resulted, and we are therefore 
compelled to regard the fact as the expression of shock. 

Theories. — Many theories have been advanced to ex- 
plain the occurrence of convulsions. No theory is entirelv 
satisfactory ; but by physiological experiments and the ex- 
periment of disease we have learned to know the conditions 
under which convulsions are most apt to occur. 

There are, first of all, the time-honored experiments of 
Kussmaul and Tenner, showing that convulsions occur in a 
rabbit after suddenly tying the cervical arteries and thus 
cutting off the blood-supply. Their conclusions are op- 



54 THE NERVOUS DISEASES OF CHILDREN. 

posed to the older theories that these seizures were due to 
an hyperasmic condition of the brain. The question arises 
whether the sudden withdrawal of blood deprives parts 
of the brain of their functions, or whether it simply acts as 
an irritant to the cortical centres. The latter explanation 
seems plausible, in view of the experiments of Hitzig, Fer- 
rier, Horsley and others. A mechanical irritation of the 
pons (floor of the fourth ventricle), as proved by Nothnagel, 
also produces convulsions. It is evident that toxic agents 
have the same effect as the electrical or mechanical irrita- 
tion just referred to, else we could not explain the convul- 
sions due to toxic poisons, those of uraemia, for instance, 
and those due to asphyxia from the accumulation of car- 
bonic acid in the blood. 

Every morbid process in the motor areas of the cortex, 
if not absolutely destructive, is apt to cause convulsions ; 
but what is the relation between the convulsive centres in 
the cortex and Nothnagel's epileptic centre in the pons? 
Does the cortex contain an actual convulsive centre? If 
you irritate the lower epileptic centre in an animal whose 
hemispheres have been divided from the rest of the brain 
you will get convulsive spasms of the entire body. The 
lower centres have the power, therefore, of " starting " con- 
vulsive seizures, and it is probable that they are under 
higher control in the fully developed brain, and as long as 
normal conditions exist. It is this power to control, this 
inhibitory force, that resides in higher centres. The re- 
moval of this inhibition through disease of such centres 
liberates the energy of the lower centres.* 

Age and Frequency. — By far the largest majority of 
convulsions occur during the first two years of life. The 
Philadelphia Health Reports, as condensed by Lewis, give 
ample proof of this. 

Seven thousand five hundred and eight deaths among 

* Neurologists, the world over, have adopted Hughlings Jackson's views without en- 
dorsing every detail of his theories. Jackson considers the central nervous system to 
be made up of three tiers of sensory-motor centres. All parts of the body are repre- 
sented in each tier, but the higher centre controls one or both below it, and has the 
power to inhibit the discharges of a lower centre. In a young child the higher tiers do 
not exert a controlling influence, since they are imperfectly developed ; hence the dis- 
charges are so much more frequent than in later years. 



C ONVUL SIONS—E CL A MP SI A INF A NTUM. 



5. 



minors (during period of 1 876-1 883 inclusive) were due to 
convulsions and laryngismus stridulus. Of these were : 





Under 

1 year. 


1 to 2 
years. 


2 to 5 
years. 


5 to 10 
years. 


10 to 15 
years. 


15 to 20 

years. 


Cases of convulsions .... 

Cases of laryngismus 

stridulus 


4,993 
24 


i,335 
7 


893 

9 


178 

5 


38 


26 






Total 


5, OI 7 


i,342 


902 


183 


38 


26 







While the statistics illustrate a general truth, some com- 
ment is necessary. First, convulsions occurring at this 
early age are not necessarily fatal, although those occur- 
ring in the first few days after birth are much oftener fatal 
than those occurring later. The brain has less power of re- 
sistance, and the processes giving rise to these early con- 
vulsions are a more serious menace to the life of the child. 

Secondly. It should not be forgotten that convulsions are 
so much more frequent during the first two years of life 
because the diseases and conditions causing convulsions are 
most frequent at this period. 

We must take into account the traumatic injury to the 
brain during labor, defective brain development, dentition, 
the acute infectious diseases, and almost all the infantile 
cerebral palsies and gastro-intestinal disorders. 

Convulsions may, however, occur at any age ; but since 
the child's brain grows less irritable the older it grows, a 
convulsion occurring later in life often signifies more serious 
trouble. 

The frequency of convulsive attacks in any given case 
will also depend upon the nature of the disease, of which 
it is merely a symptom. In the case of convulsions due to 
dentition, a convulsion or a series of convulsions may occur 
with the cutting of each tooth ; if due to gastro-intestinal 
irritation, convulsions may recur until the condition is im- 
proved or relieved. In acute infectious diseases we are 
apt to have but one initial convulsion, or at most two or 
three distinct attacks; if there were more than a few con- 
vulsions during an attack of an acute infectious disease. 1 
should suspect some cerebral complication. 

It is a serious fact, and one well worth remembering, that 



56 THE NERVOUS DISEASES OE CHILDREN. 

the initial convulsion is as little apt to be repeated as is the 
initial rigor. The first toxic invasion, and not the fever, 
seems to cause the convulsions. I have often observed 
convulsions with relatively low temperatures (103 F.) at the 
onset, without any repetition of the same during the course of 
an acute disease, even though the temperature reach 105 F. 
and over. I do not mean to deny, however, that high fever 
alone may cause convulsions, but it is not a common cause. 

I have often had occasion to observe that repeated con- 
vulsions are characteristic of cortical disease, and this is 
borne out by the cerebral diseases of childhood. 

In the majority of such cases the convulsions will be but 
one of a series of symptoms ; and if convulsions occur re- 
peatedly without any further evidences of cerebral disease, 
the suspicion of true epilepsy must be entertained. 

Symptoms. — It may seem odd that we should treat 
of the symptomatology of convulsions, which we have 
termed a symptom and not a disease, but the sequence of 
events during a convulsion is subject to great variations, 
and on this head there is need of further remark. A con- 
vulsion may be partial or general. Partial convulsions in 
the majority of the cases denote organic disease of the brain, 
and in this sense may be more serious than general convul- 
sions ; a slight convulsive twitching of a thumb may seem 
a small matter indeed, but it may be the first sign of a cere- 
bral tumor which is bound to be fatal before long ; while, as 
regards danger to life, a severe general convulsion may be 
entirely harmless. Partial convulsions are often unilateral, 
and sometimes limited to a single member. 

The cortical centre of the part convulsed is the chief site 
of the lesion. Partial convulsions often become general ; it 
is of importance, therefore, to inquire whether convulsions 
that affect all parts of the body start in any one particular 
member, or whether the person who is subject to general 
convulsions ever had unilateral partial convulsions. 

All the phenomena of a convulsive seizure need not to be 
exhibited in each attack. We may have convulsive twitch- 
ings without loss of consciousness, and loss of conscious- 
ness without twitching movements. I have clearly in mind 
a child about three months old, whom I had reason to see 



CONVULSIONS— ECLAMPSIA INFANTUM. S7 

some nine years ago. Without any known cause the child 
was in a " faint spell," during which time the skin was pale, 
and the pulse became slow and irregular. Such a spell oc- 
curred about the same hour on successive afternoons, and 
later on the child had several each day. No other symptom 
could be made out, with the exception of a distinct enlarge- 
ment of the spleen. The attacks were evidently malarial 
and yielded quickly to large doses of quinine. The child 
has had no convulsions since. 

There is some difference of opinion among authors as 
to whether a convulsive attack begins with clonic or tonic 
movements, and which preponderate. It is well to know 
that, according to the researches of Unverricht and others, 
tonic and clonic convulsions are different in degree and not 
in kind — a tonic convulsion being practically a rapid suc- 
cession of clonic movements. In children tonic spasm is 
supposed to precede clonic spasm, and in this respect to 
differ from the epileptic paroxysm of later life ; but the dis- 
tinction is of no importance, and, moreover, I have seen 
many spasms in children which were purely clonic from 
beginning to end. 

One special form of convulsive seizure in children de- 
serves special mention. This is 

Laryngismus Stridulus. — Cerebral croup, inward con- 
vulsions, child-crowing, etc. The many synonyms are in- 
dicative of the frequency of this condition, which occurs 
more often in European countries than here, owing prob- 
ably to the greater spread of severe forms of rickets in 
England and the Continent. 

In its simpler form laryngismus stridulus implies merely 
a spasm of the glottis. A child that may have exhibited an 
occasional crowing, croaking noise, is seized quite suddenly 
with severe spasm of the adductor muscles ; the body be- 
comes rigid, the head is drawn back, the face grows pale 
and then livid. In a few seconds the spasm relaxes, a deep 
inspiration follows with a hissing sound, and all is over for 
the time being, except that the child presents a haggard, 
tired look. Several such spasms may follow quickly upon 
one another. After the attack is over the child often 
vomits, is badly frightened, and from sheer fatisrue falls 



58 THE NERVOUS DISEASES OF CHILDREN-. 

into a more or less natural sleep. It is not rare to have as 
many as twenty and more of such attacks during the course 
of twenty-four hours ; in other cases single attacks follow 
each other at much longer intervals. It is quite excep- 
tional, however, to have but a single spasmodic attack. 

Other convulsive actions are apt to be associated with 
the laryngeal spasm. The diaphragm and other respiratory 
muscles are often convulsed ; the fingers are firmly clenched, 
and the toes are apt to be flexed under the feet (" carpo- 
pedal " spasm) ; general convulsive movements, and a total 
loss of consciousness may be developed before the seizure 
is over, proving the close relationship between the laryn- 
geal spasm and general convulsions." 

Causation. — There was much dispute among the older 
authors as to the true cause of laryngeal spasm. An en- 
larged thymus gland pressing upon branches of the vagus 
was supposed to be the most frequent cause; but this has 
been entirely disproven by the autopsies of Henoch and 
others, which showed that there was no correspondence 
between the state of the thymus and the occurrence of " in- 
ternal convulsions." An overpowering weight of opinion 
is in favor of a close causal relationship between rickets 
and laryngismus stridulus. The researches of Elsasser, of 
West, Gee, Gay, Henoch, Jacobi, Heubner, and many 
others have offered substantial proof of this position. Of 
late there has been a disposition to discredit this theory. 
Loos has denied the influence of rickets, while endeavoring 
to establish a relation between this convulsive disorder and 
tetany. To my mind the only proper relationship between 
the two is that they are both due, safely enough, to the in- 
fluence of rickets. Loos has been severely criticised, and, 
in fact, has been disproven, by Kassowitz, whose large ex- 
perience of rickets has stood him in good stead. 

Rachitic softening of the skull (craniotabes) has been 
supposed by Jacobi and others to be the active cause of 
laryngeal spasm. That the association of these conditions 
is unusually frequent cannot be doubted. Kassowitz states 
that only 48 of 370 cases of laryngeal spasm did not present 

* Children suffering from laryngismus stridulus are prone to the ordinary 
eclamptic fits, thus furnishing / vrurther evidence of the close relationship between the 
two conditions. 

i 



CONVULSIONS— ECLAMPSIA INFANTUM. 59 

marked craniotabes ; but of these 48 cases 47 presented 
some other decided symptom of rickets. The fontaneiles 
were closed in only 4 of these 370 cases, although no of 
these children were over one year of age. When rickets is 
not present the fontanelle should be closed at the end of 
the first year (Kassowitz). 

But is a softened skull the direct cause of laryngeal 
spasm ? Scarcely. The centres for vocal movements are 
far away from that part of the brain which is apt to be 
compressed in cases of craniotabes. The hyperaemic condi- 
tion of the brain in rickets is of a piece with the hyperaemic 
condition of other structures and it is this hyperemia 
which causes an unusual irritability of the centres which 
would not under ordinary circumstances " discharge" upon 
the slightest peripheral irritation (cold air, dentition, slight 
gastro- disturbances). Indeed there may be spontaneous 
discharge of these centres without peripheral irritation. 

Laryngeal spasms occur most frequently between the 
sixth and eighteenth months, the very period during which 
rickets is most marked, if present, though it occurs often 
enough up to the age of three years and later. Patients sub- 
ject to attacks are most apt to suffer from them during the 
coldest months of the year, viz., January, February, and 
March. 

Much has been made of the supposed relation between 
laryngismus stridulus and tetany. Cheadle believes the 
two to be identical. Facial contractility may be present in 
cases of laryngismus, but cases of laryngismus in which 
the Trousseau symptom (spasm induced by compression of 
the artery in a limb) can be elicited during the interval be- 
tween attacks are surely rare. 

There is another form of convulsive seizure in children 
which reminds one of laryngismus. Not long ago a child, 
aged eighteen months, apparently healthy, was brought to 
me that would hold its breath in many a crying spell ; after 
holding its breath for a few seconds it would turn blue, and 
its head would drop forward as it lost consciousness. After 
a few more seconds the child recovered consciousness, and 
all was well again until the next crying spell, when the 
same sequence of symptoms would be apt to occur. Treat- 



60 THE NERVOUS DISEASES OF CHILDREN. 

ment by bromides and small doses of chloral put an end to 
these attacks in a few weeks. 

Diagnosis of Convulsions. — It is scarcely credible 
that any one who has ever seen a convulsive seizure can 
mistake it for anything else. And yet it has happened 
within the author's experience that he was called to a case 
of supposed tetany, which was, however, nothing more than 
an ordinary convulsive attack. 

The difficulties of diagnosis that arise are concerned al- 
together with the possible etiological factor in a given case. 
The physician who witnesses a convulsive seizure will do 
well to be reserved in giving an explanation of the origin 
of such seizure until he has carefully examined into the 
previous history of the case ; and if he does this he will be 
able to say with great certainty whether the convulsion is 
due to some reflex irritation, to some cerebral or spinal dis- 
ease, or whether it is the initial -symptom of an acute in- 
fectious disorder. It is of the greatest practical importance, 
however, to be able to say distinctly whether the convulsion 
is the expression of functional disease or of organic disease 
of the brain. General convulsions are, in the vast majority 
of cases, of functional origin. Partial convulsions are, as a 
rule, the result of organic disease ; but a child that has gen- 
eral convulsions may at some previous stage of its history 
have exhibited partial seizures, or a general convulsion may 
have begun in a strictly localized fashion. The part first 
convulsed, or the part alone convulsed, indicates that the 
centre in the brain which governs the movements of this 
special part of the body is the chief site of the lesion. A 
twitching movement of the thumb, so slight that many 
might be tempted to regard it as a trifling nervousness, indi- 
cates disease in or near the centre governing the movement 
of the thumb. A convulsive seizure, beginning with a 
twitching of the eyelid, with the drawing up of one corner 
of the mouth, is certain to be the result of disease in the 
respective centres of these parts. 

The gravity of a convulsive seizure will depend very 
much upon the cause of the attack. In general terms a par- 
tial epilepsy pointing to organic lesions of the brain is a more 
serious matter than a general epilepsy, which may be func- 



CONVULSIONS— ECLAMPSIA INFANTUM. 6 1 

tional ; but if a general convulsion is the expression of a 
typical epilepsy about to be developed, its importance is as 
grave as though it were due to gross disease of the brain. 
Convulsions occurring at the onset of acute diseases are 
much more apt to pass off without leaving a trace behind 
them than are those convulsions which occur during the 
further course of the disease. 

There is considerable difference of opinion among au- 
thors regarding the remote dangers of convulsions. Many 
are inclined to believe that convulsions are always sympto- 
matic, and that they rarely constitute a serious danger to 
the child's life ; but others, like myself, relying chiefly upon 
a careful study of infantile cerebral palsies and of epileptic 
convulsions in the adult, are confident that convulsions are 
capable of, and often do give rise to, serious disturbance. 
This is not surprising if we consider that extreme cyanosis, 
with an accompanying intense hypersemic condition of 
the brain, occurs during the acme of the convulsion. I 
have myself recorded a case of a child dying in an epilep- 
tic convulsion, in which at the post-mortem table a wide- 
spread recent subpial hemorrhage over the convexity and 
at the base was found to have been the immediate cause of 
death. Eustace Smith refers to a case with hemorrhage 
over the base, evidently the effect of convulsions, while 
Money and others have reported cases in which a palsy, 
setting in after a convulsion, was evidently due to hemor- 
rhage from the smaller vessels of the brain coming on dur- 
ing, and caused by, a convulsive seizure. The possibility 
of such occurrences makes a convulsion a serious matter. 
It must be the earnest endeavor of the physician in attend- 
ance on the child to bring a convulsive seizure to an end as 
soon as possible. The longer a seizure lasts, the more fre- 
quently it is repeated, the greater the danger becomes — if not 
to the life of the child, at least to its future mental condition. 

Hence it follows that we must be guarded in giving a 
prognosis of the child's condition after the seizure is over. 
the majority of them leaving no unfavorable trace upon the 
child, but every now and then cases occur in which the 
convulsions were the first of a long series of disasters. 1 
shall never forget the case of a bright child who had a con- 



62 THE NERVOUS DISEASES OF CHILDREN. 

vulsive seizure at the onset of chicken-pox ; a rapidly de- 
veloping - idiocy was the result. Seizures occurring in the 
first few days after birth, inasmuch as they are the expres- 
sion of serious disease in the brain, give a particularly un- 
favorable prognosis. 

Treatment. — The first object of treatment should be to 
check the convulsion. Many remedies will have been ap- 
plied long before the physician has arrived at the bedside 
of the patient. Hot baths, mustard poultices over the spine, 
the abdomen, to the soles of the feet are still in vogue and 
urged not only by knowing mothers, but also by many ph}'- 
sicians (by Meigs and Pepper, for instance) ; but all these 
measures are to be deprecated, for they imply a loss of 
valuable time. If a convulsion ends while the child is in a 
hot bath it does so because it has run its course. 

To check the convulsion inhalations of chloroform, or of 
chloroform and nitrite of amyl in equal parts, are much to 
be preferred to other forms of medication. A few drops 
poured on a handkerchief and held to the nose of the 
child are generally sufficient to diminish the severity of the 
convulsive seizure, and the nurse or physician sitting by 
can keep up this method of administering the drug until 
the tendency to convulsions has for the time being entirely 
disappeared. Recognizing the danger from convulsions, the 
physician will do well to wait until all danger from further 
attacks has passed. After the acute stage is over small 
doses of morphia (gr. -^ to -J) or of opium, according to the 
age of the child, may be given ; and after the first day has 
been passed safely I should advise the discontinuance of 
the opiates and the substitution of small doses of bromide 
(3 to 5 grs.) several times a day, or small quantities of chlo- 
ral hydrate (gr. v. to x.), either by mouth or in the form of a 
rectal enema. 

Absolute quiet is the first essential of treatment. If 
there is good reason to believe that the convulsive seizure 
was due to gastro-intestinal irritation, or to the presence of 
intestinal parasites, steps should be taken to purge the 
bowels thoroughly, say by the use of castor-oil, calomel, 
and the like, or by appropriate treatment for the removal 
of worms. As soon as the cause has been removed, treat- 



CONVULSIONS— ECLAMPSIA INFANTUM. 63 

ment by small doses of opium, bromide, or chloral, will be 
in order for at least a week or ten days after the occurrence 
of the seizure. 

If the convulsive attack is associated with fever, there 
is of course every reason for giving a tepid bath, with sub- 
sequent cool sponging. The reduction of the temperature 
will, to a certain extent, diminish the probability of repeated 
attacks. Should a child be seized with convulsions while 
in the bath, it should be removed at once and made to in- 
hale chloroform and nitrite of amyl. It is far better in 
the cases of gastric disturbance to evacuate the stomach 
through the bowels than to attempt to give emetics, which 
of course cannot be readily swallowed, or to give hypo- 
dermic injections of morphia as suggested by Steffen. For 
to cause a child to vomit that has a tendency to convulsions 
is to increase the danger to which it is exposed. 

Cases of laryngismus stridulus can be treated on very 
much the same principle as convulsions, excepting that the 
child should not be allowed to remain in the recumbent 
position ; it should be carried about, and if breathing is inter- 
fered with, forcible means, such as pulling forward of the 
tongue, slapping of the chest with cold and wet towels, 
should be resorted to in order to bring about regular, 
rhythmical breathing. • 

If the convulsions are due to rickets, very active treat- 
ment, looking toward the improved nutrition of the child, 
will be necessary. It should be placed upon the albumi- 
noids, and only small quantities of the cereals allowed. 
Moreover, in these cases, Kassowitz's plan of administering 
phosphorus with cod-liver oil, as in the scrofulous diathesis, 
is of greatest importance. Sirup of the iodide of iron, or 
any of the many preparations of iron now to be obtained in 
the market, can be administered. If the attack is the be- 
ginning of an epilepsy, no time should be lost in placing 
the child upon the regular treatment of this condition, for 
the details of which the student is referred to the chapter 
on Epilepsy. In the cases of ursemic and other toxic con- 
vulsions, the treatment must be in accordance with the 
nature of the cause, and the condition is to be treated in 
every respect as the same condition would be treated it 



64 THE NERVOUS DISEASES OF CHILDREN. 

it occurred in the adult. If the attack is supposed to be 
due to cutting teeth, the question arises whether incision of 
the gums is apt to be of any benefit. There has been much 
discussion on this point, and yet no definite conclusion has 
been reached ; and no one, so far as I have been able to 
learn, has proved that cutting* the gums has ever injured 
the child or impaired its future health. It is well enough, 
therefore, to try this method, and personally I have little 
doubt that a temporary relief of the hypersemic condition 
of the gums is thus afforded. That the mere incision of 
the gum, as Henoch and Lewis suggest, is apt to increase 
the peripheral irritation, I can scarcely credit, if the incision 
be made according to modern surgical practice. 

BIBLIOGRAPHY. 

Cheadle : The Lancet, May, 1887. 

Chenbach : Rev. Mensuelle des Maladies de l'Enfance, 1894. 

Dana : Convulsions. Wood's Reference Handbook, etc. 

Elsasser: Der weiche Hinterkopf, 1843, p. 133. 

Gay : Brain, vol. xii., p. 482. 

Gee : St. Bartholomew's Hospital Reports, vol. iii. 

Hammond, G. M.: Medical Record, vol. xli., p. 263, 1892. 

Henoch : Vorlesungen iiber Kinderkrankheiten, 7th ed., p. 149. 

Jackson : Brain, 1886. Also his article on Convulsions in Reynolds's System, 

vol. i., p. 737, Am. edition ; Lumleian Lectures, The Lancet, March 29, 

1890. 
Jacobi: Anatomy of the Thymus Gland. Philadelphia, 1888. Also Pepper's 

System of Medicine, vol. ii. On Craniotabes, Am. Jr. of Obstetrics, 

vol. iii., 1870. 
Kassowitz : Beitrage zur Kinderheilkunde, Neue Folge, IV., p. 43. 
Lewis : Convulsions, Keating's Encyclopaedia, vol. iv. 
Loos: Deutsches Archiv fur Klinische Med.,Bd. L., 1892. 
Meigs and Pepper : Diseases of Children, p. 551. Philadelphia, 1874. 
Money: Diseases of Children, 1887. 
Nothnagel : Ziemssen's Cyclopedia, vol. xii. 
Sachs : Volkmann's Series, Nos. 46, 47, p. 452. 
Senator: Zeitschrift f. Kl. Med., Bd. VII. H. 3. (Ptomaines.) 
Smith, Eustace : Diseases of Children, p. 292 et seq. (Influence of Lead.) 
Soltmann : Gerhardt's Handbuch der Kinderkrankheiten, Bd. V., 1. 
Tarchanoff Arbt. fur Kinderheilkunde, 1879. 
Unverricht: Experimentelle u. Kl. Unters. iiber Epilepsie. Breslau, 1883. 

Deutsches Arch. f. Kl. Med., 1883, P- 44L 
West : Diseases of Children, 5th Am. ed., 1874. 



CHAPTER III. 

EPILEPSY. 

Few diseases have given rise to so much discussion 
as has epilepsy, the " morbus sacer " of ancient writers. 
Opinions regarding its cause and pathology have under- 
gone many radical changes. The causes of epilepsy were 
by most writers thought to be obscure, and it is only with- 
in very recent years that a little more light has been shed 
upon the morbid processes underlying epilepsy. Some 
claim that epilepsy is never a disease per se, always a 
symptom, while others are inclined to regard true idio- 
pathic epilepsy as a form of hereditary disease. 

Whether epilepsy is merely a symptom or a distinct 
disease the term may be used to describe a state in which 
convulsive seizures occur at varying intervals of time, and 
in which there is no special cause for each seizure. Thus 
a child may have a convulsive fit with the cutting of each 
tooth, yet we cannot say that it has epilepsy ; but if that 
same child, after dentition and its attendant troubles have 
passed, continues to have fits at shorter or longer intervals, 
the child is surely a subject of epilepsy. Fortunately, how- 
ever, only a very small proportion of those who have con- 
vulsions during childhood develop true epilepsy in later 
years. Webber reports that of 160 cases of epilepsy only 
24 began before the age of five. 

The Symptoms of epilepsy vary with the character 
of the attacks. Unfortunately the intervals between the 
attacks are not marked by. as distinct a series of symp- 
toms as is the case in tetany. In epilepsy the only symp- 
toms that we can find in the interval are the effects of biting 
the tongue, the acne resulting from the bromide treatment, 
and the general stupor and indifference which arc as often 
due to the drugs administered as to the disease itself. 



66 THE NERVOUS DISEASES OF CHILDREN. 

Epileptic attacks can be divided into two classes: 

i. Major attacks, or " grand mal." 

2. Minor attacks, or "petit mal" 

It is not uncommon to see patients who are subject to 
both kinds of attacks, and there is every possible gradation 
between the graver and lesser seizures. 

The "grand mal" attacks differ but very little from the 
convulsive seizures as described in the preceding chapter. 
It will be sufficient in this connection to enumerate in the 
order of their importance and of their occurrence the chief 
symptoms of a major attack of epilepsy. 

i. Prodromata, generally of a sensory character. At 
times there is a vasomotor or psychic disturbance. 

2. Initial cry. 

3. Loss of consciousness (very sudden). 

4. Pupils dilated ; no reaction. 

5. Tonic or clonic spasm of muscles (unilateral, partial, 
or general). 

6. Spasm of respiratory muscles, which may lead to 
asphyxia. 

7. Spasm of the muscles of the jaw (biting of the tongue, 
bloody foam). 

8. Spasm relaxes, movements become clonic and then 
intermittent. 

9. Involuntary passage of urine or of fasces. 

10. Gradual recovery of consciousness, followed by a 
prolonged stupor or profound sleep. 

In a typical attack of " grand mal " the majority of these 
symptoms will be present, but occasionally we meet with 
major attacks in which there are no distinct prodromata; 
in which the initial cry is wanting ; in which there is no 
biting of the tongue ; no relaxation of the sphincters. The 
most constant symptoms are the loss of consciousness, the 
dilatation of the pupils, the spasm of the muscles, and the 
stupor or sleep after the convulsive movements have ceased. 
As in many other diseases due weight should be attached 
to each symptom, and while the diagnosis of epilepsy should 
not rest upon a single symptom, the association of any two 
or three of the above-named symptoms will be sufficient for 
a diagnosis in any given case. 



EPILEPSY. 67 

The prodromata are of great importance. In a fair pro- 
portion of cases the patient feels that an attack is coming 
on. A vague sensation at the stomach, a feeling of numb- 
ness or of tingling in any of the extremities, are by far the 
most frequent warning signs. In other cases the aura con- 
sists of slight twitching movements, of an altered psychic 
condition, varying from a general restlessness, or irritability 
of temper, to actual maniacal excitement. In many instan- 
ces the character of the aura points to the involvement of a 
definite portion of the brain, and this is true even of cases of 
general epilepsy which are not supposed to be due to any 
gross cerebral lesion. In not a few cases temporary aphasia 
marks the coming on of an attack. 

Auditory symptoms in the prodromal stage are not un- 
common. In a little boy whom I described -in a paper pub- 
lished some years ago, every epileptic attack was preceded 
by a hissing noise like that of a steam-engine letting off 
steam. A young lady, aged twenty-five, a school-teacher, 
who was subject to epileptic attacks at every menstrual 
period, is in awe of an old woman whom she sees regularly 
before each epileptic seizure. The vision of a ball of fire, 
of the colors of the rainbow, of a sudden change in the size 
of objects, are common premonitory conditions. Still an- 
other patient perceives a very foul odor during the aura ; 
but I have never met with a case of epilepsy in which a 
pleasant or unpleasant taste preceded an epileptic fit. This 
would seem to show that the sense of taste is not inde- 
pendent of the sense of smell, although some authors refer 
in a vague manner to a gustatory aura. 

Patients who have distinct auras are on the whole 
more fortunate than those who have not, for they are able 
to prepare themselves for the attack. They can secure 
themselves against injury during the attack and may have 
time to apply remedies which either inhibit or shorten the 
spells. 

The convulsive movements should be carefully studied 
in each case, for they help to define the character of the 
epilepsy. Generally speaking, universal convulsions point 
to hereditary epilepsy. Partial or unilateral epilepsy is 
indicative of organic cerebral disease. This distinction 



68 THE NERVOUS DISEASES OF CHILDREN. 

would be a very easy one were it not for the fact that 
partial epilepsy may at any stage of the disease, and at any 
stage of an attack, become general, so that after the lapse 
of time the convulsions due to organic disease of the brain 
can in nowise be distinguished from those which are pre- 
sumably hereditary and idiopathic. It is of the utmost im- 
portance, therefore, in making the diagnosis of epilepsy, to 
inquire into the past history of the case, and to determine, 
either by personal observation or by close questioning, the 
exact manner of onset of each attack. However rapidly 
the attack may become generalized, if it begins each time 
with distinct twitchings of the thumb, of the wrist, even of 
the eyelid, there is good reason to remove such a case from 
the category of hereditary epilepsy and to range it with 
those due to organic lesions. The attacks may come on 
it definite hours of the day or night. In " nocturnal " epi- 
lepsy an abrasion of the tongue, involuntary micturition, a 
tired feeling or a headache in the morning may be the only 
evidences of an attack.* 

The typical '* minor " attacks consist of a very transitory 
loss of consciousness, without any muscular twitchings, 
without the peculiar cry, and without the involuntary pas- 
sage of urine and faeces. The loss of consciousness is often 
so slight that the child is supposed to have been " absent- 
minded " or merely " faint." Mothers and physicians, 
euphemistically inclined, are apt to speak of these attacks 
as " fainting spells." There is every reason to suspect epi- 
lepsy in any case in which " fainting spells " occur and re- 
cur without sufficient exciting cause. In some instances a 
sudden lull in the conversation of the child, a momentary 
stare, an unexpected stop when the child is practising upon 
an instrument, are the signs of petit mal ; a change in the 
child's mental condition, in its character and temperament 
(there is often increased irritability) and sometimes a mild 
form of mania, reveal the serious character of what was 
supposed to be a mere fainting spell. 

Bourneville has found a slight elevation of temperature 
during major attacks (not more than i° F.); in the status epi- 
lepticus, the condition of continuous spasms, the tempera- 

* For further details see description of convulsive attack. 



EPILEPSY. 09 

ture may rise several degrees. Thomsen and Oppenheim 
have proved the existence of a concentric limitation of 
vision, and a diminution of general sensibility for some time 
after an epileptic attack. 

The term " procursive epilepsy " has been applied to 
attacks in which the patient is suddenly impelled to run 
some distance forward, or backward ; this may be a pro- 
dromal symptom, or it may be the only manifestation of an 
attack. This variety is very rare indeed. 

We have alluded above to the psychic symptoms of 
epileptic attacks. Instead of having a typical convulsive 
seizure, the child passes into a state of mental confusion in 
which it becomes entirely irresponsible for, and unconscious 
of, its actions ; it may also pass into a condition of trance 
or into an acute mania. Prolonged periods of double con- 
sciousness are rare in children ; nor have I seen in very 
young persons the condition of narcolepsy (sudden falling 
asleep) which has been described as an occasional symptom 
of epilepsy in the adult. These " psychic equivalents " may 
be the beginning of a post-epileptic insanity. 

The mental derangement following epileptic attacks is 
often marked by violent delirium. Under the influence of 
delusions and hallucinations, the patient may be impelled 
to deeds of cruelty. Such derangement may last for a few 
hours or weeks, or it may become chronic and lead to de- 
mentia. 

Causes. — In considering the causes of epilepsy we shall 
take up, first, causes of the disease itself, and secondly, the 
causes which lead to individual attacks. That epilepsv is 
often a sad heritage can scarcely be doubted if we regard 
the statistics collected by Gowers, who finds a distinct 
family history in two-thirds of 1,450 cases. The inheri- 
tance seemed to him to come more freely from the mother's 
than from the father's side. It is also beyond dispute 
that epilepsy in the ancestry is not the only predis- 
posing cause. Other chronic nervous disorders have a 
powerful influence in this respect. I have known children 
of extremely hysterical, neurasthenic mothers to develop 
idiopathic epilepsy. Chorea in the mother is very apt to 
lead to the development of true epilepsy in the child : and. 



70 THE NERVOUS DISEASES OF CHILDREN. 

on the other hand, syphilis and alcoholism in the father 
exert a very powerful influence in this same direction.* 

According to Mendel's recent studies the majority of 
cases of hereditary epilepsy begin before the age of twenty 
years ; but up to the age of forty years idiopathic epilepsy 
may manifest itself. The same author proposes to call the 
disease " late epilepsy," if it is developed after the age of 
forty years; in twenty-five per cent, of these "late" cases 
Mendel found distinct hereditary influences. 

Accepting the truth of all these statements, I wish to 
impress one fact upon the mind of the student. Cases of 
hereditary (idiopathic) epilepsy are not nearly so frequent 
as they are supposed to be. If we examine carefully into 
the early history of our cases we shall find frequently 
that the child has either sustained some traumatic injury to 
the brain, or has acquired some cerebral lesion early in life. 
The paralysis and other symptoms which were due to the 
same lesion may have disappeared, but the epilepsy re- 
mains. I was led to this conclusion by my studies of infan- 
tile cerebral palsies, and my views have been quoted ap- 
provingly by various authors (among them Freud) who 
have become interested in the same subject. 

A very striking instance illustrative of this is that of a 
girl, about fifteen years old, who had been treated by many 
physicians for idiopathic epilepsy without any favorable 
result. On inquiring into the early history of the case I 
was told that several years previously the girl had suffered 
a slight paralytic stroke, every vestige of which had disap- 
peared with the exception of an increase of the reflexes on 
one side of the body. Testing carefully for a possible dim- 
inution of power, I found distinct traces of an old hemi- 
plegia. What was supposed to have been a case of heredi- 
tary epilepsy was clearly a case of epilepsy due to former 
cerebral disease. 

The number of cases of idiopathic epilepsy will be still 
further diminished if we exclude from the number those 
cases in which there is evidence of defective general devel- 

* Kowalewsky has insisted on the importance of syphilis in hereditary epilepsy, 
and has made a fanciful distinction between epilepsy due to paternal or maternal 
syphilis. His statements need corroboration. 



EPILEPSY. 71 

opment of the brain ; and those associated with infantile 
cerebral palsies ; for the paralysis and the epilepsy are due 
to organic disease of the brain. The cases in which epi- 
lepsy is developed after an acute infectious disease cannot 
be considered " idiopathic." 

The most characteristic cases of idiopathic epilepsy are 
those in which the disease shows itself between the tenth 
and twentieth years. A few may come on between the 
twentieth and thirtieth, but those that occur later in life, or 
in the first few years of life, should be very carefully anal- 
yzed before the diagnosis of hereditary epilepsy is made. 

It must be remembered that even in cases with dis- 
tinct hereditary predisposition some other exciting cause 
must be present to develop the disease ; thus we find that 
masturbation, disturbances of menstruation, sexual excesses, 
great emotional excitement, a blow to the head, are causes 
which lead to the development of epilepsy in those predis- 
posed.* 

Causes which Lead to Individual Attacks. — In a 
patient suffering from epilepsy any interference or disturb- 
ance of the general health of the patient may bring on an 
epileptic attack. Indigestion is perhaps the most powerful 
of these causes. Herter and Smith, in an excellent research 
on this subject, are inclined to look upon putrefactive proc- 
esses in the intestine as a frequent exciting cause of epi- 
lepsy. They do not believe that this cause w^ould be suffi- 
cient to produce epilepsy in a person not predisposed 
thereto. The same may be said of eye strain,t of urethral 

* Reflex epilepsies are described by many authors as due to peripheral injuries 
(hand or foot) ; in such cases the aura begins with symptoms referable to the injured 
part. A permanent epilepsy is rarely established in this way, and I prefer to speak 
of reflex convulsions rather than of reflex epilepsy. 

t Ranney's contributions to this subject are put forth very earnestly, but he fails to 
prove more than that the eye-conditions (errors of refraction, muscular insufficiencies, 
etc.), may cause single attacks ; but they are surely not the cause of the epilepsy. 
Moreover, every neurologist knows that by treatment directed to the improvement of 
the eye-condition the attacks may be suspended temporarily, but the disease returns 
after a varying lapse of time. Ranney's cases are for the most part reported altogether 
too early. Hern and Dodd have given but little support to Ranney's (or rather Stevens's 
views), and their statistics are open to the same criticism. Instead of reporting the 
" cures " it would be valuable to publish the later history of all c;ise;< operated upon. 
Dr. Weir Mitchell's paper concerning the history of the discovery of reflex neuroses 
may well be read in this connection. 



7 '2 THE NERVOUS DISEASES OF CHILDREN. 

stricture, of narrow prepuces, and of laryngeal irritation. 
I doubt whether any one of these conditions has ever been 
the sole cause of epilepsy, though I am willing to concede 
that they may be sufficient to produce attacks in persons 
with this special hereditary taint. 

Improper oxygenation of the blood is another frequent 
cause of attacks. This explains why so many epileptics 
have their seizures in crowded court-rooms, in theatres, and 
in poorly ventilated bedrooms. The irritation of the epi- 
leptic centres in the lower portion of the brain is directly 
responsible for these attacks. After allowing for all these 
various causes we are compelled to admit that many of the 
epileptic seizures occur at more or less regular intervals, 
for reasons that we cannot discern, though I am firmly 
convinced that the more carefully we regulate the life and 
habits of the epileptic patient the fewer attacks he will 
have. It is very largely a question of removing all excit- 
ing causes. 

A few powerful drugs have been known to cause epi- 
lepsy. Heiman described a number of cases of epilepsy due 
to poisoning by cocaine, and Tuczek has dwelt upon the 
potent influence of antipyrin. In the case of children 
cocaine need not be feared ; but the careless exhibition 
of antipyrin might be responsible for unpleasant occur- 
rences. 

Gray has called attention to the frequent occurrence 
of epileptic attacks in those suffering from chorea and mi- 
graine. I have seen several patients, in advanced years, 
in whom the epilepsy followed upon the cessation of mi- 
graine. 

Diagnosis. — The diagnosis of epilepsy offers but little 
difficulty if the character of the individual attacks has been 
clearly made out, and if such attacks recur at more or less 
regular intervals. 

The epileptic nature of a seizure is determined by the 
presence of some of the several symptoms which go to make 
up a complete attack. The chief difficulty will be experi- 
enced in differentiating between an epileptic seizure and an 
ordinary fainting spell ; also between epilepsy and hysteri- 
cal attacks. 



EPILEPSY. 



n 



The characteristic features of each attack will be found 
in the following tables : 



Epileptic Attacks. 

Loss of consciousness very sudden. 
Warning of short duration. 

Pupils dilated ; do not contract to 

light. 
Pulse unaltered. 
Tonic and clonic spasms in various 

parts of the body. 
Bloody foam at mouth. 
Involuntary passage of urine and 

faeces. 
Prolonged stupor after attack. 



Fainting Spells. 

Loss of consciousness gradual. 
Warning of some minutes before 

consciousness is lost. 
Pupils contracted or unaltered. 

Pulse feeble. 
No spasms. 

No evidence of biting of the tongue. 
No involuntary passage of urine or 

fseces excepting in rare instances. 
Recovery prompt after attack. 



Epileptic Attacks. 

Loss of consciousness sudden and 

absolute. 
Warning of short duration. 

Pupils dilated. 

Tonic and clonic spasm. 



Eyes turned upward and inward. 

Involuntary passage of urine and 

fasces. 
Prolonged stupor. 



Attacks at rarer intervals. 
Duration of attack short. 



Hysterical Attacks. 
Loss of consciousness not absolute. 

The attack often preceded by emo- 
tional excitement. 

Pupils not dilated. 

Tonic rigidity ; exaggerated conscious 
movements ; arching of back ; ex- 
cessive noises. 

Eyes staring, not turned, sometimes 
closed. 

No involuntary passage of urine or 
faeces. 

Recovery gradual ; no stupor. The 
patient may pass, however, into a 
trance condition. 

Attacks may be frequently repeated. 

Duration of attack much longer. 



The distinction between organic and idiopathic epilepsy 
can easily be made if we simply call to mind that organic 
epilepsies are, as a rule, partial, if not unilateral. Idio- 
pathic epilepsies are invariably bilateral or general in their 
manifestations. 

If organic disease is suspected, the manner of onset of the 
individual attack should be carefully determined, for partial 



74 THE NERVOUS DISEASES OF CHILDREN. 

or unilateral manifestations often become general during 
the attack. Under the head of organic epilepsies we must 
include post-kemiplegic epilepsy. This form comes on with 
deplorable frequency after the paralytic attacks in early 
life. The epileptic movements as a rule affect the paral- 
yzed side, but after the disease has lasted for some years 
the child, although its paralysis is unilateral, is prone to 
have general epileptic attacks. Under these circumstances 
the relation between the general epilepsy and the hemi- 
plegic form of paralysis can scarcely be doubted. It will 
not be amiss to insist again upon the fact that the evidence 
of an existing hemiplegia may be so slight that it will be 
overlooked unless specially examined for; but however 
slight such traces may be, if it can be shown that the epi- 
lepsy was developed after the onset of the hemiplegia there 
is good reason to suppose that both the paralysis and the 
epilepsy are due to the same organic lesion. I consider it 
a safe rule in epilepsy beginning in childhood to examine 
particularly for exaggeration of the reflexes ; and an in- 
crease of the reflexes in one-half of the body is quite as safe 
a sign of a preceding hemiplegia as a marked paralysis with 
contracture would be. 

The sudden onset of epileptic movements in a child pre- 
viously healthy should lead one to suspect the possibility of 
intra-cranial tumor ; and a slight weakness of the parts con- 
vulsed, a possible increase of the deep reflexes in that same 
part, the presence of headaches and the development of 
optic neuritis, are the symptoms that we must look for in 
order to establish or to discard the diagnosis of tumor. 

The same series of symptoms may occasionally be pres- 
ent in cases of acquired or hereditary syphilis ; if so they 
are the result of general specific infiltration of the brain 
coverings, or of the development of gumma together with 
this general infiltration. 

Prognosis. — The prognosis of epilepsy, whether of the 
organic or idiopathic order, is altogether bad. This con- 
viction deepens the larger one's experience with this di- 
sease. Do what we will, the attacks recur as soon as treat- 
ment is abandoned. I have seen but very few cases of 
absolute cure of genuine epilepsy. One was the case of a 



EPILEPSY. 75 

young girl, now twenty years old, in whom the disease was 
developed at the age of twelve, coincident with the onset 
of menstruation. She has been free from attacks for very 
nearly five years. I have known the attacks to be inhibited 
for a year, and even for two years, and yet they have re- 
turned after that period of time if the customary treatment 
was stopped. In this respect there does not seem to be a 
wide difference between organic epilepsy and idiopathic 
epilepsy, excepting that in the case of the former an early 
termination in death can be looked for if the attacks are due 
to malignant disease. The frequent development of de- 
mentia, idiocy, or of epileptic insanity, in epileptics makes 
the outlook gloomier still. In spite of all recent therapeu- 
tic efforts we have not been able to accomplish anything in 
the way of a radical cure of the disease. The only ray of 
hope that we have is that in the beginning of the disease 
the attacks may be due to special conditions which do not 
necessarily imply the existence of hereditary epilepsy. The 
fond hope that the child may outgrow the tendency to epi- 
leptic seizures is rarely realized. 

Pathological Anatomy. — Our knowledge of the mor- 
bid changes in epilepsy is very incomplete. Meynert laid 
great stress upon induration of the cornu amonis. Chronic 
hydrocephalus has been found in many cases, but this can 
hardly be considered a cause of epilepsy, for if it signifies 
anything it merely implies that the same process which led 
to the development of hydrocephalus also caused the epi- 
lepsy. 

It is much more probable that the morbid changes of 
true epilepsy will be revealed through a study of the corti- 
cal changes which give rise to a localized epilepsy in cases 
of traumatic injury to the brain, and of epilepsy associated 
with infantile hemiplegia. In these conditions the initial 
stages of a morbid process* leading to epilepsy can best be 
studied ; and it is possible, if not probable, that general epi- 
lepsy has such a beginning at a time and in a part of the 
brain unknown to us. Marie, Fere and Chaslin have led the 
way in these studies, and report the discovery of a neuroglia 

* For other morbid processes associated with epilepsy see chapters on Cerebral Pal- 
sies and on Idiocy. 



76 



THE NERVOUS DISEASES OF CHILDREN. 



wmmmm 

mMmm 






sclerosis which they consider to be the cause of epilepsy — 
such sclerosis starting from a focus of disease. 

Van Gieson has made a very valuable contribution to this 

subject by a study of cortical 
tissue, removed by McBurney, 
from two cases of Starr. Van 
Gieson demonstrates most sat- 
isfactorily the changes in the 
large pyramidal cells of the cor- 
tex and in the neuroglia. He 
finds that " the ganglion cells 
are affected by a series of de- 
generative changes, which in 
their most advanced stages re- 
sult in an almost complete dis- 
solution of the cell, and yet this 
degeneration is not extensive 
enough to involve the cells so 
universally as to interfere with their topographical distri- 
bution." 

Some of the degenerative changes of the cells will be 

evident in the figure below taken from Van Gieson's article. 

There is a decided change — a true hyperplasia — in the 






Fig. 39. — Diffuse Neuroglia Sclerosis 
of the Cortex in Epilepsy (after 
Chaslin). 




Fig. 40. — Various Phases of the Earlier Stages of the Degeneration of the Ganglion 
Cells. The thin lines enclosing the cells w and u represent the pericellular spaces ; 
the cells x and y show the earliest stages, w and s later stages, and k shows the 
ultimate destruction of the whole of the ganglion-cell body, leaving nothing but the 
nucleus lying in an empty space. (Van Gieson.) 



EPILEPSY. 77 

neuroglia tissue ; clusters of an increased number of very 
young and seemingly proliferating neuroglia cells are vis- 
ible in the vicinity of the small pyramids. 

These findings were very much the same in two cases : 
in one the condition was due to a foreign body, and in the 
other an old cicatrix seems to have induced a similar path- 
ological state in the neighboring tissue. To appreciate 
such researches at their true worth, it is important to note 
that they have reference to the early conditions only. What 
the nature of the secondary changes is, and how these are 

m 1 #■'*£» ja 



§) 






Fig. 41. — A Group of More Mature Neuroglia Cells in the Layer of Small Pyramids. 

(Van Gieson. ) 

developed from the original forms of disease is a subject for 
further study. It will be a fertile field of research for any- 
one who undertakes with the ever-improving histological 
methods, to study the entire brains of epileptic children and 
adults. 

Pathology. — The pathology of the epileptic attack is 
identical with that of convulsive seizures in general. It is 
not necessary therefore to repeat in this chapter what was 
said in the chapter on convulsions. The onlv question of 
importance is whether in the diseased brain the convulsive 
centre on the floor of the fourth ventricle plavs as impor- 
tant a role as it does in physiological experiments. Bins- 
wanger found that electrical stimulation of the lower halt 
of the floor of the fourth ventricle in animals would pro- 
duce tetanoid attacks or spasm in the limbs and the trunk, 
but he did not succeed in causing an ordinary epileptic at- 



78 THE NERVOUS DISEASES OF CHILDREN. 

tack. There can be very little doubt that in man an epi- 
leptic attack is in the vast majority of cases the result of 
cortical irritation. The character of the sensory auras is 
evidence of this, and so is the observation made by Oebeke 
and Gowers, that epileptic attacks cease after a lesion in 
the internal capsule.* 

As for the pathology of the disease itself, opinions have 
undergone a very marked change within the last few years, 
owing to the rather startling suggestion of Pierre Marie, that 
epilepsy is often of infectious origin. There is much in 
favor of this view, but we must not attach undue impor- 
tance to the element of infection, and should remember that 
the convulsions which occur so frequently in the acute fe- 
brile diseases of childhood give rise to serious vascular dis- 
turbances, and that a focus of disease, due to purely me- 
chanical causes, may be the beginning of general changes 
throughout the brain, which are responsible for the per- 
manent establishment of epilepsy. 

Treatment. — In discussing the treatment of epilepsy it 
is best to proceed on the theory that the attack is due to 
over-action of the cortical cells. Gowers explains the influ- 
ence of the bromides by stating that " if we regard the 
morbid state in epilepsy as an instability in the resistance 
of the nerve-cells, it seems probable that the effect of the 
bromide is to increase the stability of that resistance." 
Whatever the explanation may be, we have up to this time 
found no drugs that can in any sense be considered proper 
substitutes for the bromides. f 

It is certain that very few cases of epilepsy have been 
permanently cured by the administration of the bromides ; 
but unquestionably they serve an admirable purpose in 
checking the number of attacks and in diminishing their 

* Horsley pleads strongly for the invariable cortical origin of the epileptic attack, 
and believes that during the attack the cortex is in a congested rather than an anaemic 
condition. It will help us very little to say that the attacks are due to toxine poi- 
soning. This may or may not be true ; but in every case the most important ques- 
tion to settle is, why should certain individuals be prone to attacks and others not, 
while these same toxines from intestinal putrefaction, for instance, are present in 
hundreds of others who never have an epileptic seizure. 

t A recent writer explains the action of the bromides by their power to diminish 
the effect of the toxines circulating in the body. If toxines and bromides were in- 
jected into the veins of an animal at one and the same time, the result was less 
severe than if the toxine alone was injected. 



EPILEPSY. 79 

severity. To accomplish this end the bromide salts should 
be administered according- to a definite plan. It has been 
my practice to give preference to the bromide of sodium, 
which I employ, according to the age of the patient, in ten 
or fifteen grain doses, three times a day. If given in a 
wineglassful of (alkaline) water after meals, the gastric 
functions will not be seriously impaired. Erlenmeyer sug- 
gested, some years ago, that a combination of the three 
salts — the bromide of sodium, the bromide of ammonium, 
and the bromide of potassium — would answer far better 
than the exhibition of any one of these salts singly. He 
went so far as to claim that if a single salt had produced 
acne, that that acne would disappear upon the administra- 
tion of the three salts combined. I have given this method 
a fair trial, but have not been able to convince myself of 
the truth of Erlenmeyer's claim. The bromides should be 
pushed in every case to the point of tolerance, and until 
the attacks have been successfully diminished or inhibited. 
The loss of the palate reflex is evidence that the patient 
has become thoroughly brominized, but in children intol- 
erance to the drug is very apt to be established long before 
this point has been reached. 

The bromides can be administered in divided doses, two 
or three times a day ; but if there is no good reason to 
give the drug during the day, there is a special advantage 
in giving the entire daily dose shortly before bedtime. The 
stupefying effect of the drug is not felt as distinctly as 
when it is administered during the daytime, and the sound 
sleep that ensues is an additional advantage to the patient. 
In the case of nocturnal attacks the medicine should be 
given before going to bed, and at no other time. In his 
recent admirable lectures on the treatment of certain func- 
tional neuroses Seguin advocates the plan of giving the 
bromides on very much the same principle that one would 
adopt in giving quinine in malaria. In other words, the bro- 
mides should be given according to the periodicity of the 
attacks, giving little in the interval and increasing the dose 
very much at the time when an attack is expected, or if 
the attacks are frequently repeated, to give the drug four 
to five hours prior to the time at which the seizures occur. 



80 THE NERVOUS DISEASES OF CHILDREN. 

I can subscribe thoroughly to this recommendation, as it has 
done me admirable service in the treatment of epileptic at- 
tacks in children. If the tendency to attacks has been over- 
come, the drug should be continued for a period of at least 
one year after the last attack. The dose may be dimin- 
ished gradually, but it is wise to keep the patient in a 
state of mild brominism. If it is desirable to diminish the 
amount of bromide the patient takes, the good effect of the 
drug can be maintained by giving, in conjunction with the 
bromide, five to ten grains of chloral hydrate, a few minims 
of the tincture of digitalis, or of the tincture of belladonna. 
Innumerable other drugs have been suggested ; among 
these I will mention cannabis indica, which is particularly 
effective in cases of epilepsy with chronic headaches. The 
oxide and lactate of zinc have been freely recommended, 
but I have seen no beneficial results. The administration 
of iron and arsenic is thoroughly rational ; no doubt the 
restorative effect of these substances upon the blood is 
the indirect cause of improvement in the epileptic con- 
dition. Some years ago Gowers advised the use of fif- 
teen to thirty grains of borax several times daily. A fair 
trial has been given this drug in my clinic and in private 
practice, but I cannot claim more for it than for dozens of 
other drugs.* Flechsig has lately advised a combination 
of opium and bromides. Opium is given for six weeks ; 
beginning with one-half to one grain ; the dose is increased 
gradually until the patient takes eight, ten, or fifteen grains 
daily ; then it is stopped suddenly, and the bromides in 
thirty-grain doses, four times daily, are substituted ; after 
some time the dose is diminished to five or ten grains per 
day. I was ready to adopt this suggestion, as I had for 
many years been using codeine together with the bromide 
salts. In children the doses must be somewhat altered, 
but the plan of treatment, according to my own experience, 
deserves further trial, f 

* Dr. Joseph Collins has summarized the more recent measures suggested in the 
treatment of epilepsy. He has a kind word to say for borax, for nitroglycerine (in 
cases of vasomotor aura), and for Flechsig's method; but utterly condemns the use 
of osmic acid, simulo, hydrastinum, and other drugs. 

t While this chapter was going through the press Bechterew has advised a combi- 
nation of bromides and Adonis vernalis. 



EPILEPSY. 51 

The treatment of epilepsy is by no means exhausted by 
the recital of a few medicinal agents. Success depends 
upon other factors. The greatest possible attention should 
be paid to the details of the patient's daily life ; regularity 
in the hours of sleep, in the hours of meals, and careful 
dieting, are essential to proper treatment.* I place my epi- 
leptic patients upon a general mixed diet, including small 
quantities of albuminoids and a liberal vegetable diet, but 
I forbid all indigestible articles of diet. I also limit the 
amount of cereals, and try to check intestinal fermentation 
as far as possible, being moved to this by the result of Her- 
ter's researches. Pastry and sweets of all sorts are strictly 
prohibited. Fresh air at all times, and particularly at night, 
should be insisted upon. Freedom from all emotional ex- 
citement is another recommendation that should not be 
overlooked. Marked ocular defects and other peripheral 
conditions which may cause an epileptic attack, are to be 
treated. 

Older patients who have distinct warning of their epi- 
leptic attacks should be provided either with the pearls of 
nitrite of amyl, which they can break up and inhale at short 
notice, or else they can be given a mixture of equal parts of 
chloroform and nitrite of amyl, which can be carried in a 
small phial and can be poured upon a handkerchief for 
purposes of inhalation as soon as the attack is signalled. 
In the case of partial epileptic attacks of definite onset a 
ligature applied around the part first convulsed, to be tight- 
ened quickly as soon as the warning comes, has been rec- 
ommended by Hughlings Jackson, and is serviceable in 
some cases. 

Surgical Treatment. — The surgical treatment of epi- 
lepsy has attracted great attention during the past ten 
years or more, ever since it has been known that the motor 
centres in the cortex can be safely and easily reached 
by the surgeon's knife. Surgical interference should be 
considered only in cases of partial epilepsy pointing to a 
definite focus of disease as the starting-point of the entire 
trouble. Nothing seems easier than to expose the centre 

* To carry out these measures successfully, I am now in the habit of placing such 
patients under the rest cure whenever feasible. 
6 



82 THE NERVOUS DISEASES OF CHILDREN. 

of the part first convulsed in an epileptic attack, to remove 
this centre, and thus to remove the seat of disease, but, un- 
fortunately, our ardent hopes in this matter have not been 
realized. 

Some years ago Dr. Gerster and myself reported the 
results of operation in ten cases of epilepsy. The cases 
were carefully selected, and if surgical operation could 
have been of avail in any case of epilepsy, it would have 
been so in those we selected for operation ; we were bound 
to admit that in our experience some slight improve- 
ment followed, but also that not a single absolute cure 
had been effected. Since that article was written my ex- 
perience has been fully doubled, and the conclusions reached 
are practically the same. An analysis of the cases reported 
by Starr, which included my own cases, does not justify one 
in taking a more hopeful view of this question. I have 
referred in former writings to the reason for this disap- 
pointment. After an initial injury to the brain, months, 
and sometimes years, elapse before the epileptic habit is 
established. In about the same length of time a general 
cerebral sclerosis has been developed in connection with 
the original focus of disease. We may remove the orig- 
inal focus, but the general sclerosis remains and will act 
as a constant irritant upon the remaining epileptic centres. 

On the other hand, it would be hasty to conclude that the 
surgeon's skill can be dispensed with in these cases. * The 
aim should be to watch carefully for the development of 
epilepsy, and to remove injured tissue at the earliest pos- 
sible date. More than this, I consider it important not to 
await the actual development of epilepsy ; and if the brain 
has sustained any considerable injur}^ to remove the in- 
jured tissues, which, if allowed to remain, constitute a per- 
manent menace to the future health of the patient. We 
shall be able to prevent the development of epilepsy very 
much more readily than we can cure it if once established. 

The surgical procedures in vogue at the present day for 
the cure of epilepsy are trephining and excision of diseased 
tissues. Trephining has been practised for centuries. It 

* The author wishes to direct particular attention to the excellent work done by 
Kocher. 



EPILEPSY. 83 

is a method that entails no special danger upon the life of 
the patient operated upon, and can therefore be tried with 
perfect impunity except in very young children. White, 
of Philadelphia, and others believe that its only effect is 
the same as that of any operative procedure ; but I am 
inclined to think that if it has any good effect it is in the 
way of relieving increased pressure, and that increased 
pressure is more frequently a real factor in epilepsy than is 
generally supposed, on account of the frequency of cystic 
formations and of the increased ventricular fluid in a very 
large proportion of the cases. Excision of tissue would 
seem to be a rational procedure in the earlier stages of 
epilepsy developed after traumatic injury, or in connection 
with early cerebral disease; but the paralysis which so 
easily results from the excision of the motor centres mil- 
itates somewhat against the advisability of this practice, 
although according to Dana's and my own experience such 
paralysis disappears after a few weeks. In cases in which 
the epileptic attack is preceded by sensory or psychic 
phenomena, a removal of the auditory or visual centres, 
for example, would be a serious matter indeed, if not en- 
tirely unjustifiable. Many a patient would prefer the oc- 
casional repetition of an epileptic attack to a permanent 
loss of hearing or a permanent impairment of sight. 

The only other* surgical procedure which has been sug- 
gested for the cure of epilepsy is the ligature of one or 
both vertebrals, as was first done by Dr. Alexander, of Liv- 
erpool. The dangers of this operation are so great, and 
the results so uncertain, that it scarcely merits serious con- 
sideration. 

BIBLIOGRAPHY. 

Bidwell and Sherrington : British Medical Journal, 1893, November 4, p. 988. 

Binsvvanger : Arch. f. Psych., 1888. 

Bourneville ; Recherches sur l'Epilepsie, l'Hysterie, etc. Paris, 1893. 

Blocq-et-Marinesco : (Pathology) Sem. Med., 1892, p. 445. 

Collins : New York Medical Record, September 22, 1894. 

Dodd : Brain. Winter Part, 1893. 

* I do not consider it necessary to refer to the surgical treatment of the eve-muscles 
for the cure of epilepsy except utterly to condemn the practice. As I am treating of 
epilepsy in children, I need not discuss oophorectomy and similar procedures. 



84 THE NERVOUS DISEASES OF CHILDREN. 

Echeverria : Epilepsy. New York, 1870. 

Erlenmeyer: Behandlung d. Epilepsie, Monograph., pp. 513, 573. Berlin, 
1892. 

Fere : Les Epilepsies. Paris, 1890. 

Fischl : Zeitschr. f. Heilkunde, 1890. 

Flechsig : Neurologisches Centralblatt, 1893, p. 229. 

Francois, Frank : LeQons sur les Fonctions du Cerveau. Paris, 1887. 

Fraenkel : Beitrage zur Chirurg. Festchr. fur Th. Billroth, 1892. 

Gerster and Sachs : American Journal of the Medical Sciences, November, 
1892. 

Goldstein : Schmidt's Jahrb., vol. ccxliii., p. 100. 

Gray : Keating's Cyclopaedia. 

Heiman : Dtsch. Med. Wochenschr., 1889. 

Hern : British Medical Journal, September 30, 1893. 

Herter and Smith : New York Medical Journal, 1892. 

Horsley ; British Medical Journal, April, 1892. 

Kocher : Zeitschr. fur Chirurgie, 1892; also 1893, xxvi., pp. 1-93. 

Kowalewsky: Syph. Epilepsie, Berl. Kl. Wochenschr., 1894, No. 4. 

Kiimmel : Dtsch. Med. Wochenschr., 1893, pp. 326 and 531. 

Kussmaul and Tenner : Publications of Sydenham Society, 1859. 

Marie, Pierre: Sem. Med., July, 1892. 

Mendel: Neurolog. Centralbl., 1893, p. 664. 

Nothnagel : Article " Epilepsy " in Ziemssen's Cyclopaedia ; also Wiener 
Medicinische Presse, 1893. 

Parker and Gotsch : British Medical Journal, May 27, 1893. 

Reynolds : Monograph, London, 1861 ; also in Reynolds's System of Medi- 
cine, Article Epilepsy. 

Ranney : New York Medical Journal, 1 893 and 1 894. 

Sachs : New York Medical Journal, February 20, 1892. 

Seeligmuller : Dtsch. Med. Wochenschr., 1 and 2, 1894. 

Seguin : Functional Neuroses, New York Medical Journal, 1890. 

Starr : Brain Surgery, 1893. 

Tuczek: Berl. Kl. Wochenschr., 1889. 

Trowbridge : Al. and Neurologist, February, 1892. 

Van Gieson : New York Medical Record, April 24, 1893. 

Webber : Boston Medical and Surgical Journal, 1893, vol. xxviii., p. 491. 

Weir Mitchell : Medical News, Philadelphia, April 28, 1894. 



CHAPTER IV. 

HYSTERIA. 

True hysteria is a relatively rare condition in adults. 
This may seem a very heterodox statement to those who 
have been ready to apply the term hysteria to many affec- 
tions of the nervous system which could not be attributed 
to organic lesions. Those who are advised of the recent 
conception of hysteria will not hesitate to indorse the au- 
thor's opinion. The determination of the chief symptoms 
of true hysteria, and the separation of this disease from 
many other functional disturbances which were once classed 
with it, have constituted a signal achievement in modern 
neurology. We have at last succeeded in establishing a 
number of symptoms, chiefly of a sensory character, which, 
when present, enable one to make the diagnosis of hysteria 
with a great degree of certainty, and which do away with 
the necessity of reaching this diagnosis by the process of 
exclusion. As the physician's knowledge of nervous dis- 
eases increases, the diagnosis of hysteria is made more and 
more infrequently. 

We see much less of true hysteria in this country than in Europe ; it is 
also far less common in England and in Germany than in France and Rus- 
sia. 

In the author's very large clinical service at the New York Polyclinic the 
diagnosis of hysteria is one of the rarest of all diagnoses made. In other in- 
stitutions with which the writer is connected, particularly in the Montefiore 
Home for Chronic Invalids, hysteria is found to be much more frequent, 
especially among the foreign-born inmates. Broadly speaking, the Anglo- 
Saxon race is less prone to the development of hysteria than the other races 
represented in our population. 

If hysteria is a rare disease in the adult, it is still rarer 
in the child, but when it does occur it is an important 



86 THE NERVOUS DISEASES OF CHILDREN. 

factor in the future life of the child. Many of the writers 
on diseases of children have neglected this subject entirety, 
and others have given but scant notice of it. The older 
English and American authors have had very little to say 
upon the subject. Meigs and Pepper, as well as West, 
scarcely refer to it. In the large hand-bock of Gerhardt, 
Jolly has treated the subject in a very satisfactory manner, 
and in American Cyclopaedias and Systems of Medicine 
the subject has received careful attention at the hands of 
Mills and Lloyd. Henoch has written a very full chapter 
on the subject, but includes under this heading many forms 
of disease which are more properly designated by other 
names. The French authors have naturally had much to 
say on the subject, and they include so many different forms 
under this heading that one is puzzled to know whether 
there is any form of nervous or mental disease that is not 
in some way related to hysteria or influenced by it.* 

The symptoms of the disease are truly protean in char- 
acter. No one person, fortunately, ever exhibits even a 
majority of them. Hysteria in one person is very different 
from the disease as it is exhibited in others. It is difficult, 
therefore, to give any one clinical picture of the entire dis- 
ease. It will be best to take up the chief symptoms as they 
occur independently of other diseases, or as they are known 
to complicate other functional and organic diseases of the 
nervous system. Hysteria is characterized by symptoms 
which point to a defect in the various systems and organs 
of the body. Thus we have : 

i. Psychic manifestations. 

2. Motor manifestations. 

3. Sensory manifestations and vasomotor disturbances. 
1. Psychic or Mental Hysteria. — Properly speaking, 

every case of hysteria would come under this heading. 
For, if there is any one feature that distinguishes hysteria 
from other diseases, it is the defect of will-power and the 
excess of the emotional faculties. " I can't " is the pet 
phrase of all hysterical subjects, or still better, " I will not." 
It is not so much a direct lack of power to exert the will, 

* Moebius proposes to designate as " hysterical " all those morbid physical states 
which are engendered by concepts. 



HYSTERIA. 87 

as a tendency to exert it in perverse fashion. This peculiar 
mental condition is easily recognized, and often leads to the 
diagnosis of hysteria in persons who have other symptoms 
pointing to a much more serious disease. But aside from 
this general hysterical state of mind there are other mental 
conditions which are very typical of hysteria. The most 
pronounced psychic form of hysteria, as observed in chil- 
dren, is that known as hysterical mania. Under great men- 
tal strain or excitement a child is seized with a crying or 
laughing spell, after which it passes into a state of nervous 
excitement in which, as in a little girl, aged eleven, under my 
observation, the child becomes violent, attempts to strike 
others, to injure herself, and to tear her clothes from the 
body, and to do all possible injury irrespective of conse- 
quences. 

Another condition, sometimes classed under the heading 
of hysteria, is observed in young girls, particularly at the 
age of puberty, and often ends in a condition of marked 
acute mania. I have seen a number of such cases in girls 
who were over-ambitious and eager to pass their school ex- 
aminations. They would keep up under the excitement of 
the examination, but immediately thereafter, whether suc- 
cessful or not, would become irritable, excitable, sleepless, 
would have laughing and crying spells by turns, would re- 
fuse to take nourishment, and eventually would either re- 
cover under proper treatment, or else pass into a condition 
of typical acute mania with absolute loss of reason, with in- 
tense excitement, and confused delirium. There would be 
no reason to consider these cases in this connection if they 
did not occur in children who have shown either a marked 
nervous predisposition, or who have exhibited hysterical 
symptoms of one kind or another at previous periods. 
Such children, if subjected to severe strain or severe emo- 
tional excitement, are very apt to pass into a condition of 
hysterical mania. 

Other mental conditions which cannot well be separated 
from hysteria, are those known as hystero-epilepsv, cata- 
lepsy, hysterical trance, and the like. Of these, hystero- 
epilepsy is by far the most important, and the gravest dis- 
order. Mills is inclined to consider this form of disease 



88 THE NERVOUS DISEASES OF CHILDREN. 

very rare among children. In this he is undoubtedly cor- 
rect; but it has been my good fortune to see a number of 
classical instances of this special form in children, and the 
account I give is based entirely upon personal impressions. 
The gravity of hystero-epilepsy is increased by the fact 
that the children so afflicted are met with in mentally de- 
generate families. Insanity, epilepsy, chorea, chronic alco- 
holism, are the predisposing conditions in the ancestors of 
those who suffer from hystero-epilepsy. In one family I 
have during a period of ten years treated the mother for 
severe hysteria, a daughter at the age of nine for hysteri- 
cal convulsions, and another son and daughter for typical 
hystero-epilepsy. 

On account of this close relationship to true epilepsy, 
cases of hystero-epilepsy cannot be regarded with the in- 
difference which many physicians still display toward hys- 
terical subjects. In many cases it is difficult to decide 
whether the attacks as they occur are more hysterical or 
more epileptic ; and of the attacks occurring in one individ- 
ual some may be typically hysterical, while others may be 
typically epileptic ; and there is some danger in every case 
of severe hystero-epilepsy that with the progress of years 
the hysterical symptoms may vanish and true epileptic at- 
tacks may occur. It is of the utmost importance, before 
passing judgment upon any case, to determine whether the 
attacks are epileptic or hystero-epileptic. 

2. Motor Manifestations. — Every variety of clonic 
and tonic movements occur in hysterical subjects. Many 
of these will be understood best by referring in detail to a 
few cases of typical hystero-epilepsy. 

Some seven years ago I was called to see a lad, then 
thirteen years old, who had been seized with violent con- 
vulsions during the night. I had been informed that these 
convulsions were preceded by great emotional excitement, 
caused by a severe upbraiding which the boy received for 
his misbehavior. After this little domestic scene was over 
the boy went to bed, and was seized with the first attack. 
During the attack he gave a shrill cry ; then began to bark 
like a dog, snapping at everyone who approached him, and 
would pass through the most severe contortions, touching 



HYSTERIA. »9 

the bed at times only with the head and heels, the back be- 
ing- deeply arched as in the famous drawings of Richer 
representing this condition in women. After this the most 
violent jactations of the entire body occurred. During 
these convulsive movements he would snap, bark, and bite, 
then he would suddenly give a leap to the foot of the bed — 
almost tumbling out of bed — yet he always saved himself in 
time. This whole performance would last about two or two 
and a half minutes, then thorough relaxation of all the mus- 
cles would take place, he would fall back exhausted, and 
would then regain consciousness. During the attack there 
was no evidence of consciousness, at least no impression 
could be made upon him through any of the special senses. 
The patient often had as many as two hundred such attacks 
in the course of twenty-four hours ; he was sleepless, refused 
food, and became very much emaciated. After careful nurs- 
ing for a period of six weeks the attacks gradually lessened, 
and eventually he recovered entirely ; but he has shown since 
a deficiency in his moral and mental make-up, and although 
he has been cured of the hystero-epilepsy, it was found 
necessary to place him in a reformatory. This last fact is 
of some interest as showing the relation which hystero- 
epilepsy bears to degeneration of all the mental and moral 
faculties. 

Another case was that of a young girl, aged fourteen, 
who was much worried over the fear that she and the family 
would starve, as the father had daily drummed into the 
child's ears the necessity of economy and the difficulties of 
supporting a family in hard times. The young girl, the* 
daughter of a very hysterical and emotional mother, took 
the warning to heart, and for the first time in her life de- 
veloped typical hystero-epileptic attacks. Without warning- 
she would fall from a chair, from a sofa, or even on the 
street would pass suddenly into a condition of apparent un- 
consciousness. Wherever she lay she would pass through 
all sorts of contortions, would shriek, now and then would 
give agonizing yells, and would finallv end up by a pro- 
nounced crying spell. At other times she would become 
violent, tearing her own clothes, the bed-linen, and every- 
thing that was within her reach in the room. These spells 



9 o 



THE NERVOUS DISEASES OF CHILDREN. 




HYSTERIA. 



91 



were repeated very frequently during a period of three 
weeks, and then gradually subsided. During all these at- 
tacks the movements seemed to be more or less purposive, 
and yet there was good reason to think, and the girl later 
on confessed, that she was absolutely unconscious of what 
she was doing at the time. These two cases will suffice as 
a general indication of the common form of hystero-epilep- 
tic attacks. 

The distinction between the attacks of epilepsy and of 
hystero-epilepsy is brought out in the following table : 



Epilepsy. 

Aura frequent. 

No distinct cause for each attack. 



Hystero-Epilepsy. 

No aura ; but, 

Some emotional excitement, direct 

cause of attack. 
Onset sudden or gradual. 
Noises of all sorts during attack. 



Onset sudden. 
Initial cry. 

Eyes open or closed ; pupils often Eyes turned up ; pupils normal ; 

often ecstatic expression of coun- 
tenance. 

Either tonic rigidity of muscles or 
extravagant movements (some- 
times purposive). 

No impairment of vesical and rectal 
reflexes. 

Attacks last much longer, followed 
by a condition of trance, or else 
patient recovers consciousness as 
soon as convulsive movements 
cease. 

The patient falls softly, without per- 
sonal injury. 

Biting of the tongue rare. 

Patient may pass into condition of 
trance, or may exhibit signs of fa- 
tigue ; recovery often rapid. 



dilated widely ; do not react ; roll- 
ing of eyes upward and inward. 
Tonic and clonic convulsions. 



Involuntary micturition or defeca- 
tion. 

Duration of attack only a few min- 
utes. 



The patient often injures himself. 

Biting of the tongue. 
After cessation of clonic movements, 
stupor or somnolence. 



In addition to the typical attacks of hystero-epilepsy 
there are other forms of motor disturbance due to hysteria. 
The hystero-epileptic attack, while it is the gravest, is in fact 
much rarer than the ordinary hysterical convulsion. The 
latter is characterized by a temporary and imperfect loss 
of consciousness ; by irregular, though sometimes violent, 
twitchings of all extremities, sometimes by the repetition 



92 THE NERVOUS DISEASES OF CHILDREN. 

of one special form of convulsive movement, such as re- 
traction of the head. When these various convulsive move- 
ments are over, the muscles pass into a state of tonic rigid- 
ity, after which rapid recovery takes place. The entire 
episode is wound up by a fit of crying or laughing, or by 
some other expression of an emotional character. Some of 
the patients pass into a condition of melancholy which may 
last for hours, or into a state of exaltation and even of ec- 
stasy. This is as true of the hysterical attacks as they oc- 
cur in children as it is of those that occur in the adult. 
These attacks can be distinguished from the hystero-epilep- 
tic and from epileptic seizures by the incomplete loss of 
consciousness, by the absence of all regular rhythmical 
convulsive movements, and by the fact that there is neither 
biting of the tongue, nor involuntary micturition, nor any 
of those symptoms which are often associated with hystero- 
epileptic and epileptic attacks. Other hysterical attacks 
consist of spasm of the head and neck muscles, the well- 
known spasm of the oesophagus which gives rise to the 
sensation known as globus hystericus, or to difficulties in 
deglutition if the spasm affects the lower part of the gul- 
let. This oesophageal spasm which occurs in hysterical 
and otherwise neurotic children is a symptom which has 
not met with the appreciation which it deserves. If a 
sound is passed into such a gullet it will be seen that there 
is an actual muscular spasm at the point of constriction, 
and that it requires considerable patience, and sometimes a 
little force, to overcome the contraction ; but the age of the 
child and the fact that the constriction easily disappears, to 
return again under the slightest emotional excitement, is 
sufficient to remove any fear of the constriction being due 
to an organic cause. In this same category we may place 
spasmodic movements of the diaphragm, of which singultus 
is the common manifestation. This special phenomenon is 
much more frequent in later periods of life than during the 
period of childhood ; but 1 have had under observation an 
entire family afflicted with this special form of hysteria 
who upon the slightest provocation would exhibit this 
symptom. On one occasion I examined the mother, in the 
presence of two daughters, respectively twelve and ten 



HYSTERIA. 93 

years of age. Under the excitement of the examination 
the mother was seized with severe singultus, and before I 
had completed the examination both the daughters were 
favoring me with a similar exhibition. The scene would 
have been a comical one if it had not brought home to me 
the powerful influence of example and suggestion, if not of 
heredity, in such cases. 

Spasm of the bladder, increased peristaltic action of 
the stomach and bowels resulting in diarrhoea, upon the 
least emotional excitement, fits of crying or laughing, of 
sneezing and of coughing, are the various forms of hyster- 
ical spasm met with in children, as well as in persons of 
more advanced age. If the hysterical seizure, or better 
said, the hysterical spasms, assume a more extravagant 
character, and if it affects a larger number of the groups of 
muscles that generally act in unison with one another, we 
may have that peculiar condition which is known as cJiorea 
major. In this form the jactations are often severe and ex- 
hausting. The child or young person assumes the most im- 
possible positions, often suggesting the intention of dra- 
matic effect. From this condition it is but a short step to 
a condition of mild tonic contracture, the limbs retaining 
any position which may be given them. This state of cata- 
lepsy is very often witnessed in conjunction with chorea 
major, sometimes independently of it, but it is a mistake 
to assume that catalepsy occurs exclusively in conjunction 
with hysteria. It occurs in connection with various forms 
of mental derangement, and a condition not unlike cata- 
lepsy is met with in some forms of infantile cerebral paraly- 
sis. 

In hysteria the muscles of the body are not only sub- 
ject to convulsive movements but also to absolute paral- 
ysis. There is not a part of the body that may not be 
the seat of such palsy, but it is most frequently mani- 
fested in the extremities, in the tongue, and in the vocal 
cords (hysterical aphonia). In some instances there is no 
absolute paralysis of any one muscle or group of muscles, 
but certain functions are paralyzed. The best known ex- 
ample of this is the form which has recently been described 
as astasia-abasia. A person thus afflicted is neither able to 






94 THE NERVOUS DISEASES OF CHILDREN. 

stand nor to walk, but is perfectly well able to use all mus- 
cles while lying on the back. Many have claimed that this 
is simply due to a psychic condition dominated by the 
idea that standing or walking is impossible. This is in all 
probability the proper interpretation ; but the point that 
concerns us here is that it rarely, if ever, occurs except in 
those who exhibit other symptoms of hysteria. 

Hysterical aphonia is very frequent in children, and par- 
ticularly during the period of development. The hysteri- 
cal character of the aphonia is easily recognized by the fact 
that (as the laryngoscope reveals) there is no organic cause 
for the loss of voice, and that the single attacks come on 
very suddenly, as a rule, in the wake of some nervous ex- 
citement, and disappear as rapidly as they have appeared. 
I have had young girls come to my consulting-room who 
would not be able to speak above a whisper, and it has al- 
ways been a special pleasure to have them sing the entire 
scale before leaving the room. A strong faradic brush ap- 
plied to the neck over the trachea is the most persuasive 
master in these cases. Hysterical mutism, an absolute loss 
of speech, is generally the result of severe emotional ex- 
citement. In some instances it is associated with delusions, 
and with other symptoms of mental derangement. 

Other forms of hysterical paralysis are not so easy to 
diagnosticate, and in order to be able to differentiate them 
from paralysis due to organic causes the physician must 
have all neurological facts and doctrines at his command. 
It is indeed one of the most difficult problems in neurology 
to distinguish hysterical from organic forms of paralysis, 
but this distinction can be made safely enough if the fol- 
lowing points are kept in mind : Hysterical paralysis does 
not as a rule adhere to anatomical distribution, with the one 
exception that an hysterical hemiplegia may be quite as 
complete as any hemiplegia due to an organic disease. But 
I have not yet met with a single case of hysterical hemi- 
plegia in a child that has in any way suggested, even for a 
moment, the possibility of a hemiplegia due to an organic 
lesion in the brain. In hysterical paralysis the paralysis, as 
well as the anaesthesia which is associated with it, is apt to 
be regional ; thus we have a paralysis of the hand, or a pa- 



HYSTERIA. 95 

ralysis of hand and forearm, or paralysis of an entire extrem- 
ity, with an anaesthesia that, as a rule, develops in propor- 
tion to the paralysis. This association of regional anaesthe- 
sia and regional paralysis is, to my mind, very characteristic 
of hysteria. 

From cerebral palsy, hysterical paralysis can be distin- 
guished by the fact that it is not accompanied by increased 
reflexes ; and by the lack of marked sensory changes in the 
majority of cases of brain paralysis. The deep reflexes may 
be lively in cases of hysterical paralysis, but I have never 
seen them markedly exaggerated, nor are they accompanied 
by the spasticity and tonic contractures which are so fre- 
quent a symptom in the case of paralysis due to brain dis- 
ease. The presence of ankle clonus was at one time sup- 
posed to prove the non-hysterical character of an affection ; 
this may be true in the majority of cases, but since I have 
met with the presence of ankle clonus in some hysterical, 
and even in neurasthenic, affections, I cannot attach much 
importance to this one point. The electrical reactions, it 
should be remembered, are normal both in hysterical and 
in cerebral diseases ; we cannot therefore utilize them in any 
way in making the differential diagnosis. 

If the question arises whether paralysis of the upper or 
lower extremities (more frequently of the latter) is due to 
spinal disease, or whether it be purely hysterical, the diag- 
nosis should be based upon the absence of vesical and rec- 
tal symptoms in a case of hysterical paralysis, though to be 
sure there are many cases of spinal disease too in which 
these are not affected. Retention of urine may occur in 
cases of hysteria, but it is only of short duration as a rule, 
and is rarely accompanied by the symptoms of cystitis, as is 
the case in a large number of spinal palsies. In many cases 
of spinal and peripheral nerve disease the electrical reac- 
tions are altered, and the reaction of degeneration may be 
present. This is not the case in hysteria ; but these elec- 
trical reactions are normal also in all those cases of spinal 
disease which are due to disease of the lateral columns and 
not of the posterior columns, or of the central gray matter. 

All these points of differential diagnosis may in some 
cases prove insufficient, and the diagnosis of hysteria or of 



96 ' THE NERVOUS DISEASES OF. CHILDREN. 

organic disease of the nervous system will depend upon the 
general agreement or disagreement of symptoms. If the 
physician is aware that flaccid paralysis of a single group 
of muscles, or of one or more extremities, is generally as- 
sociated with changes in the electrical reactions, and with 
loss of reflexes in case these symptoms are due to organic 
disease, and if he remembers, furthermore, that spastic forms 
of paralysis are associated with increased reflexes, with nor- 
mal electrical reactions, and with normal sensations, he will 
have little or no difficulty in arriving at a correct diagnosis. 
Hysterical paralysis is subject, moreover, to greater changes 
than the organic palsies are. While under certain condi- 
tions the very persistence of the symptoms for a long period 
of time, followed by a sudden change, is extremely char- 
acteristic of hysterical paralysis, the diagnosis becomes 
still more certain if the hysterical paralysis is associated 
with other symptoms, such as are known to be purely hys- 
terical. I refer particularly to the presence of rigidity, 
anaesthesia, or typical and complete hemianassthesia, and to 
the presence of major or minor hysterical attacks ; but the 
difficulties are still further increased by a fact to which 
Seguin called particular attention some years ago, that 
hysterical paralysis or hysterical symptoms often compli- 
cate organic disease. 

A characteristic case is that of a young girl, aged thirteen, who some 
years ago had acquired a typical poliomyelitis. The sudden onset, the in- 
volvement of the anterior tibial group, the flaccid and atrophic paralysis, 
the presence of the reaction of degeneration, the absence of the knee-jerk 
placed this diagnosis beyond a doubt. Two years after the onset of the 
poliomyelitis the young girl was seized with a complete paralysis of the fore- 
arm and hand of the same side. The arm could be raised a little at the 
shoulder with flexion at the elbow ; flexion at the wrist or any movement of 
these parts was impossible. There was at the same time an anaesthesia ex- 
tending from the tips of the fingers of this right hand to a line drawn around 
the arm at about one and a half inch below the elbow. Every form of 
sensation was absent in this part. The hand and forearm showed no ten- 
dency to atrophy, the electrical reactions were entirely normal, but the hand 
remained stationary in the position resembling that practised by the accou- 
cheur (a favorite position in hysterical subjects). There was no increase of 
the reflexes in the upper extremity, and there were no symptoms pointing to 
any organic lesion, either of the brain or spinal cord. There was no reason 
therefore to hesitate in making the diagnosis of hysterical paralysis, and I 



HYSTERIA. 97 

have on many occasions exhibited this young girl to my students as an ex- 
ample of a patient who exhibited both an hysterical and an organic affec- 
tion. 

Another case will illustrate the same point. A young girl, aged fifteen years, 
was brought to my clinic, with a history of a fall upon the arm, causing a 
dislocation of the head of the humerus, a year previously. The arm was set, 
but marked paralysis, with excessive pain along the median and ulnar 
branches, remained. The pain disappeared, but in spite of every form of 
treatment the paralysis would not yield. The girl was surrounded by an 
over-sympathetic mother and very loving sisters, who insisted on providing 
her with pillows and all sorts of appliances in order to protect her against 
pain, and the arm against injury. The family had made her a confirmed in- 
valid. At the time of my examination I found that the arm was but very 
little atrophied, that there was no objective disturbances of sensation, that 
the electrical reactions had returned to an almost normal state, the faradic 
and galvanic excitability of the median and ulnar nerves still being somewhat 
diminished ; but the galvanic reactions of all the muscles were good, their 
contractions were prompt, and the formula was not altered in any muscle, 
yet the paralysis stubbornly persisted. It was evident that an hysterical 
form of paralysis had been superimposed upon a paralysis which was origi- 
nally due to a peripheral neuritis. The separation from the family, the en- 
couragement given her by a competent nurse, the use of electricity and of 
hydro-therapeutic measures, brought about a most marvellous improvement 
in less than two weeks — a change which could never have been effected so 
quickly by these same remedies if the paralysis had not been of an hysterical 
character. 

French authors have reported a number of cases of extreme progressive 
wasting of muscles in hysteria. Hirt has recorded a case in a young girl, so 
astounding in the rapidity and degree of development of the atrophy that it 
almost challenges belief. 



3. Sensory Symptoms. — Disturbances of sensation con- 
stitute a very characteristic symptom in the majority of 
cases of hysteria. As has been intimated in the preceding 
pages the sensory symptoms often enable one to make a 
positive diagnosis of the hysterical character of the affec- 
tion when other symptoms would leave some room for 
doubt. Every form of sensation may be affected, and it 
may either be exaggerated or diminished. Hysterical 
hyperassthesia and hysterical anaesthesia are of common 
occurrence. If hyperassthesia exist there is an unusual 
sensitiveness to the slightest touch or to the slightest pain- 
ful impression. This hyperesthesia is most prominently 
developed in the region of the ovaries, in the skin over the 

7 



98 THE NERVOUS DISEASES OF CHILDREN. 

spinal column, each spinous process being so sensitive to 
touch that the patient cries out from pain, however light 
the touch may be. The hyperaesthetic areas, if stimulated, 
are very apt to cause distinct hysterical attacks in those 
prone to such seizures, and the hypersesthetic areas may 
become true hysterogenic zones. These hysterogenic 
zones have been studied with great care by the French 
school, but we cannot enter upon the subject more fully 
here, and leave it with the simple statement that there are 
some hysterogenic zones which are not hypersesthetic. 

Hysterical anaesthesia is still more frequent than the 
exaggerations of sensor)' impressions. The anaesthesia 
may not only include every form of ordinary sensory per- 
ception but also the special senses. The distribution of 
this anaesthesia is one of the most valuable signs of hysteria. 
It occurs in the form of a complete hemianaesthesia, or in 
the form of a regional anaesthesia. The hemianaesthesia is 
limited strictly to one-half of the body, but in this half it 
is often absolute, and the completeness of the anaesthesia is 
the very feature which should make us suspicious of its 
hysterical nature. If a girl or a boy, or for that matter any 
person at any age, present absolute anaesthesia to all forms 
of ordinary sensation, and in addition exhibits blindness of 
one side or a typical hemianopsia, deafness, loss of taste, 
and of smell in the same half of the body, such a person 
has undoubtedly hysteria. In this country hysterical 
hemianaesthesia is relatively rare, excepting in the Russian 
and French elements of our population, but in these it can 
be studied easily and satisfactorily. 

If an entire half of the body is not anaesthetic, the re- 
gional character of an anaesthesia is sufficient to lead one to 
the diagnosis of hysteria. By regional anaesthesia I mean 
anaesthesia of a well-marked division of the body, say of 
the hand, of the forearm, of the leg, the hips, or of circum- 
scribed areas in any part of the body without reference to 
the anatomical distribution of the sensory nerves. (Fig. 43.) 
As this regional anaesthesia is frequently associated with a 
regional paralysis the hysterical nature of both becomes 
very evident. The hysterical nature of the anaesthesia is 
not only made evident by its peculiar manner of develop. 



HYSTERIA. 



99 



ment, but also by the manner in which it often disappears. 
In a number of cases in which I have studied this anaesthesia 
carefully it would recede inch by inch, but always main- 
tained a certain level in the arms or legs without any ref- 
erence to the well-known sensory areas. Hysterical pa- 
tients do not present anaesthesia of the anterior or posterior 
surface of a limb, but the anaesthesia generally involves 
both surfaces. The hysterical nature of sensory disturb- 
ances is also established by the powerful effect of hypnot- 




FlG. 43.— The Three Types of Distribution of Anaesthesia in Hysteria : Hemianaes- 
thesia, Segmental, and Disseminated. Z, Hysterogenic Zones. (Dana.) 

ism, and of mere suggestion, which is often sufficient at 
least temporarily to dislodge a complete anaesthesia. The 
anaesthesia may furthermore be transferred from one limb 
to another, not merely by the action of magnets, or bv the 
action of metals as was formerly supposed, but, I am con- 
vinced, by any form of suggestion powerful enough to pro- 
duce a strong psychic impression. It is on the theory of 
suggestion that we must explain the miracles ascribed to 
magneto-therapy or metallo-therapy, that played such an 
important role some ten or fifteen years ago in France. 

Of the special senses vision is most frequently subject 
to hysterical disorder. In some there is true photophobia. 
in others a diminution of visual perception is more common. 



100 



THE NERVOUS DISEASES OF CHILDREN. 



and the patient may indeed be unaware of the existence of 
such diminished visual sensation, which is the best proof 
that it is not intentional or simulated. The retina may be 
entirely insensitive to light, there may be distinct limita- 
tion of the visual field, or there may be a complete loss 
of every form of visual perception in one eye. (Fig. 44.) 
Bilateral loss of sight is said to occur in hysterical patients, 
but as a rule it is simply transitory. I have not had op- 
portunity to see this special visual defect in children. 

Visceral hysteria deserves a passing notice ; the paral- 
ysis of the bladder and increased peristaltic action of the 




o. s. 



O. D. 



Fig. 44. — Hysterical Loss of Color Sense and Limitation of Visual Field. Color 
Sense Absent in Left Eye and Field Contracted ; in Right Eye Field less Con- 
tracted ; Order of Appreciation of Colors from Without In was Yellow, Violet, Blue, 
Red, Green. (After Peck, from Dana.) 



stomach and bowels have been mentioned in connection 
with the motor disturbances of hysteria, but there are two 
other forms of hysterical manifestations that are worthy of 
further mention. The one form is hysterical anorexia. 
Patients in this condition absolutely refuse food, and if they 
take it, vomit it at once. In some instances this is accom- 
plished without effort on the part of the patient and seems 
entirely unintentional. In other instances, again, the pa- 
tient deliberately sets to work to eject the contents of the 
stomach, and does not give up the effort until she has suc- 
ceeded. In my service at the Montefiore Home I frequent- 






HYSTERIA. 10 1 

ly had occasion to witness the antics of an hysterical girl, 
aged fifteen, who immediately upon taking food would go 
into a corner of the room, begin violent gagging movements, 
and would succeed within a period of three or four minutes 
in ejecting the entire contents of the stomach. In this girl, 
who was not possessed of any insane delusion regarding 
the character of the food, the act seemed purely volitional, 
and it was a surprising fact that like many other hysterical 
patients with anorexia, she was not so much reduced in her 
physical condition as a person would have been who had 
persistently starved herself. She evidently obtained food 
and retained it ; but how she got it and when she took it 
it was difficult to determine, since she was watched care- 
fully night and day. She was treated by rather heroic 
measures in the form of forced feeding and hydro-thera- 
peutic measures ; she was allowed to leave the institution 
after she had shown her willingness to take food and re- 
tain it. 

Distention of the stomach and bowels, representing a 
total paralysis of the muscular walls of the stomach and in- 
testines, occurs frequently enough, and more particularly in 
children. The stomach is apt to be distended to an enor- 
mous degree, and the bowels dilate in such a way as to give 
the appearance of the abdomen in a case of large-sized 
tumor. I have in mind the cases of twin brothers who pre- 
sented the following peculiar condition : The one brother, 
at the age of fourteen, was true to the neurotic stock from 
which he descended by suddenly developing a tremendous 
meteorismus, which increased from day to day until the 
abdomen was so distended that the skin seemed ready to 
burst. There was some anorexia and constipation. The 
constipation was occasionally relieved, with the result of 
temporarily diminishing the distention of the bowels, but 
in a very few hours the intestines would resume their for- 
mer state. Every possible measure was resorted to, to re- 
move this distention, but it persisted stubbornly for some 
weeks, when finally, after the introduction of large enemata 
of an infusion of valerian, the distended abdomen suddenly 
collapsed. A few days later the twin brother of this patient 
began the same performance, and went through exactly the 



102 THE NERVOUS DISEASES OF CHILDREN. 

same experiences. The abdomen became distended to almost 
the same degree as in the brother, and after it had lasted 
about the same length of time his abdomen also collapsed in 
very much the same way. These boys not only came from 
very neurotic stock, but had been very much pampered in 
their early training, which fertilized the soil that had been 
prepared for the development of every form of hysteria. 

Diagnosis. — It would be impossible to state the points 
of differential diagnosis between hysteria and other affec- 
tions without repeating everything that has been said in 
the preceding paragraphs. It is worth while stating once 
more that the diagnosis of hysteria should be made only in 
case an organic affection can be positively excluded, and if 
the well-recognized symptoms of hysteria, particularly hys- 
terical seizures and hysterical sensory changes are present. 
Let the student also bear in mind that hysteria and hypo- 
chondriasis are not one and the same disease, though one 
is often mistaken for the other. If a boy is fearful of a dis- 
ease with which he supposes he is afflicted, we have no 
hesitation in saying that he is a hypochondriac ; but let a 
girl exhibit exactly, the same symptom, and she is at once 
put down as an hysterical subject. To distinguish between 
the two conditions is not always an easy matter, but in 
hypochondriasis the patient is generally possessed of a few 
notions regarding his own bodily condition, and these make 
such a deep impression upon him that his entire ego is af- 
fected by it. His supposed affliction is constantly before 
his mind, and dominates his entire being. In cases of hys- 
teria there is no such introspection as in hypochondriasis. 
The bodily ailments or bodily peculiarities are far more 
numerous, but they are subject to greater changes, disap- 
pear for a time and then return again, and are not pushed 
to the fore quite as much as in cases of hypochondriasis. 
In the latter, moreover, the depression is, as a rule, greater 
than in hysteria, and in hysteria there are other symptoms 
which prove the presence of an hysterical affection. The 
difficulties of a differential diagnosis are still further in- 
creased by the fact that a mixture of the two conditions 
(hysterical hypochondriasis) is not uncommon, and it must 
be left to the physician to determine, by a close analysis 



HYSTERIA. 103 

of the symptoms, whether there is more hysteria or more 
hypochondriasis in the symptoms which the patient pre- 
sents. 

Pathology. — In discussing the pathology of hysteria 
there is much room for theorizing, but there are very few 
facts to guide us. That it is a functional disease is con- 
ceded on all sides; but there is no other disease in which 
the loss of function may be so absolute as in hysteria. By 
some mechanism or by some influence which we cannot 
yet understand, an entire hemisphere is temporarily invali- 
dated, or else we could not explain the typical and com- 
plete hemianassthesia so common in hysteria. Meynert 
endeavored to give an anatomical explanation, but failed. 
How this loss of function of one hemisphere is effected, and 
whether it is similar or not to the occurrences that take 
place under hypnotic influence, we are not prepared to say. 
The transfer of sensory disturbances from one-half of the 
body to the other, would go to show that the two halves 
of the brain are evidently in sympathy with one another 
(to put it broadly) in this disease, and that they can be al- 
ternately affected. The highest centres are evidently im- 
paired in hysteria, cortical inhibition is removed, and the 
lower centres have full sway.* It is quite in keeping with 
this view that even the reflexes are frequently exaggerated 
in hysteria as they are in organic disease in which the 
changes in the motor fibres of the pyramidal tract interfere 
with the proper transmission of cortical influences. We 
cannot properly speak of the morbid anatomy of hysteria, 
for the entire conception of the disease would have to be 
altered if we could suppose the existence of such. Some 
post-mortem findings have been reported, but they were 
evidently accidental complications. 

Duration and Course. — Once hysterical always hys- 
terical, would seem to be the general opinion with regard 
to these subjects, but this is not quite accurate. The mani- 
festations of this disease often disappear for years, and it is 

* Breuer and Freud have attempted a psychological explanation of hysterical phe- 
nomena and have ascribed these to a " hypnoid " condition in which there is a division 
of consciousness, and a limitation of the power of association. Freud has directed at- 
tention to the diminution in the strength of concepts in hysterical subjects, and to the 
ready change from psychic to physical innervation. 



104 THE NERVOUS DISEASES OF CHILDREN. 

one of the commonest experiences to record the disappear- 
ance of an hysterical paralysis or of an hysterical anaes- 
thesia. The tendency to relapses is extremely great, and 
symptoms that have disappeared for years may occur un- 
der any emotional excitement. 

Treatment. — The treatment of hysteria must be con- 
sidered with reference to the hysterial predisposition and 
with reference to the special hysterical symptoms. The 
hysterical predisposition is unfortunately either inherited 
from a neurotic ancestry or it has been specially fostered 
by an hysterical environment. In hysteria, as in other men- 
tal affections, I have often doubted whether heredity is the 
marked factor that it is generally supposed to be, or, if we 
grant the hereditary tendency, whether the hysterical man- 
ifestations would be as frequent as they are if a serious at- 
tempt were made to change the surroundings of such chil- 
dren. In the majority of cases the early training has been 
defective, and an example constantly before the child of an 
hysterical mother or other hysterical relatives is sufficient 
to engender the disease in its fullest form. The first and 
most important principle of treatment, therefore, is the ab- 
solute separation of the child from the family. It is a great 
pity that this is so rarely urged by the physician, and still 
more rarely permitted by the parents. Only the more in- 
telligent parents can be made to understand that an utter 
stranger, if properly qualified, may train a child far better 
than its own mother can. At all events there is nothing in 
the treatment of hysterical children as important as plac- 
ing them under the influence of a sober-minded, intelligent 
nurse or teacher who will devote herself or himself to study- 
ing the peculiarities of the child, and who will make a seri- 
ous attempt to foster the good qualities and to counteract 
the vices. If this course that has been suggested is per- 
sisted in, it should be followed not for weeks or months but 
for years, and I am confident that if this is done during the 
formative period of a child's mind and character, an excel- 
lent result will follow in many instances. I base this upon 
my own experience, and have often stated to parents that 
if they will not allow me to pursue such a plan of treatment 
I prefer not to attempt any. 



HYSTERIA. 105 

The Weir-Mitchell treatment, which is so effective in 
many cases of adult hysteria, is also of the greatest value in 
the hysteria of children ; but I consider it to be one of its 
special advantages that it presents the first and easiest op- 
portunity for the separation of the child from its immediate 
surroundings. Select a nurse carefully, place the child en- 
tirely in her charge, and many a parent will soon be con- 
vinced of the good that comes from such separation. In 
addition to the moral influences which may be exerted in 
this way the physical health of the child can be looked 
after. Bad habits of diet and of general hygiene can be 
corrected, and a child whose general physical condition 
has been far below par up to the time of its entering upon 
the rest cure, may be turned into a strong and vigorous be- 
ing. Drugs, I am very certain, have but little influence 
over such conditions. The usual hysterical remedies, such 
as asafcetida, valerian, and the like, may be employed, but 
they will accomplish little which cannot be accomplished 
by other means as well. If the effect of a drug depends 
upon its disagreeable taste, suggestions of a different char- 
acter will prove quite as efficient ; and above all the phy- 
sician should avoid the possible risk of engendering the 
idea in a growing child that there is a special drug which 
it may resort to for any annoying or painful sensation. 
There is nothing more disgusting than the habit so freely 
practised by many physicians of giving valerian or asafce- 
tida or morphine to children or adults, whenever they pre- 
sent symptoms which smack of hysteria, or which cannot 
be interpreted otherwise by the attending medical adviser. 

The special symptoms of hysteria call for distinct forms 
of treatment, but whatever these special symptoms may be, I 
always consider it wise to bear the general hysterical condi- 
tions in mind, and to employ, in addition to the special treat- 
ment, those general measures which have an excellent invigo- 
rating effect upon the nervous system. I refer more particu- 
larly to the proper use of hydrotherapeutic measures. The 
treatment of hysterical aphonia was referred to above. Sim- 
ple faradism is all that is needed in most instances, and if this 
is not sufficient, regular vocal exercise in the hands of an in- 
telligent teacher or nurse, will bring about the desired effect. 



106 THE NERVOUS DISEASES OF CHILDREN. 

I have never found it necessary to apply the faradic current 
to the inside of a throat, but of course would not hesitate 
to do so in case the external application did not prove suf- 
ficient. Blistering or the use of the static machine act as 
powerful forms of local suggestion. In hysterical paralysis 
of the limbs, the use of a strong faradic current is gener- 
ally the most effectual remedy. The current applied know- 
ingly, not mercilessly, will gradually induce the patient to 
attempt similar contractions ; and if to the use of the faradic 
current be added the encouraging words of the nurse or 
physician, who should not, however, accuse the child of 
simulation, and if other measures, such as massage, be em- 
ployed, the hysterical paralysis will disappear in the course 
of time. But many of the cases are extremely stubborn 
and may require more vigorous measures, or more power- 
ful suggestion, before they yield to treatment. It is of 
some importance at times to prove to the child that it can 
use its limbs very much better than it supposed. Place it 
in the middle of a room quite by itself, make it stand or 
crawl or walk, and if once shown that it can do so, the para- 
lytic symptoms may rapidly disappear. But the treatment 
of hysterical patients of all ages and of all classes demands 
unusual tact and patience on the part of everyone con- 
cerned in the treatment of the case. 

The sensory disturbances of hysteria are best influenced 
by the use of the cold douche or of the faradic current, 
particularly by the employment of the faradic brush. If 
such measures as I have suggested are not sufficient to re- 
move an hysterical paralysis or an hysterical anaesthesia 
the effect of suggestion, hypnotic or otherwise, may be 
attempted. 

The visceral disturbances common in hysteria also de- 
mand special treatment. Some of the measures to be em- 
ployed were referred to in connection with the hysterical 
twins mentioned above. In cases of hysterical anorexia 
patient efforts should be made to induce the patient to take 
small quantities of food, and if these are not retained forced 
feeding must be resorted to ; but never, if you can avoid it. 
resort to rectal feeding, for the patient who has discovered 
that she can be fed in that way will continue to refuse food 



HYSTERIA. 107 

very much longer than she would otherwise. Lastly, the 
treatment of hysterical attacks may be managed in very 
much the same way as other hysterical manifestations are 
treated. First of all, the patient should receive the general 
anti-hysterical treatment, and should be given some drug 
which will act as a powerful irritant or a powerful form of 
suggestion when the attack is imminent. 1 have found 
nothing better than a sudden douche of cold water, or cold 
flagellations on the head and chest, or the inhalation of ni- 
trite of amyl. The latter has this to recommend it, that it 
may be used with all the more assurance in those cases in 
which the suspicion of epilepsy cannot be altogether ex- 
cluded. Lavender and ammonia are also efficient in some 
cases in which the hysterical attack can be inhibited by any 
such simple measure. If an hysterical attack continue for 
a prolonged period of time it may be brought to an end 
by the brisk use of cold douches, by the application of a 
strong faradic current, or by pressure over the ovaries, 
after the age of puberty, if these organs be oversensitive. 



BIBLIOGRAPHY. 

For detailed information see the text-books of Grasset, Henoch, Hirt, 
and Gowers. 
Babinski : Atrophy with Hysterical Palsies, Arch, de Neurologie, July, 

1886. 
Bourneville : Progres Medical, 1880. 

Breuer u. Freud: Neurologisches Centralblatt, 1893, Nos - l and 2 - 
Bussard : Archiv der Phys., 1887 ; Neurolog. Centralbl., 1893, I. and II. 
Bruns : Review of Traumatic Forms, in Schmidt's Jahrb., 1892 and 1893. 
Charcot : Lecons du Mardi a la Salpetriere. Paris, 1889. And in numerous 

other publications : Progres Me'dical, 1882. 
Dana : American Journal of the Medical Sciences, 1892. 
Freud: Neurologisches Centralblatt, 10 and 11, 1894. 
Gillette : New York Medical Journal, 1882 (in a child of eighteen months). 
Gilles de la Tourette : Treatise on Hysteria. Paris, 1S91. 
Hirt: Deutsche Med. Wochenschr., 1894, Extreme Muscular Atrophy in 

Hysteria. 
Jacobi : American Journal of Obsterics, 1876. 
Janet : Archives de Neurologies, 1893, XXXV.-XXXVI, 
Jolly: In Ziemssen's Cyclopaedia, and Berl. Kl. Wochenschr.. 1892. No, 34. 
Lowenfeld : Neurasthenie und Hysteric Monograph. Wiesbaden. 1804. 



108 THE NERVOUS DISEASES OF CHILDREN. 

Mills : Article in Keating's Cyclopaedia (with ample references). 

Moebius : Ueber d. Begriff d. Hysterie, Centralblatt fur Nervenheilkunde, 

1888 ; also Neurologische Beitraege. Part I. 
Remak, E. : Berl. Kl. Wochenschr., 1892. 
Riesenfeld : Hysterie bei Kindern. Inaug. Diss. Kiel, 1887. 
Tuczek: Berl. Kl. Wochensch., 1886, No. 31. 
Weir Mitchell : Lectures on Nervous Diseases. 



CHAPTER V. 

CHOREA. 

Among the neuroses of childhood none is worthier of 
careful study than chorea. The name is made by some to 
include a number of varying conditions, but its use should 
be restricted to designate a functional disease characterized 
by irregular, involuntary twitchings of some or all of the 
muscles of the body. These movements cease, as a rule, 
during sleep. 

Synonyms. — This neurosis is also known as St. Vitus's Dance and the 
Chorea of Sydenham. Both these names deserve to be used, since the former 
implies its historical origin, and in the latter is preserved the name of the 
famous English physician who first described its most characteristic symp- 
toms. Scelotyrbe and Melancholia saltans, are terms occasionally used, 
though almost obsolete. In German text-books the disease is called Chorea 
Minor, in contradistinction to Chorea Major, a neurosis of a purely hysterical 
character.* 

Etiology. — Chorea is distinctly a neurosis of childhood 
and early adolescence. The vast majority of cases begin in 
very early youth, though Sinkler, a few years ago, reported 
two cases in persons over eighty years of age. Careful sta- 
tistics have been gathered with reference to this disease, 
the most elaborate being those reported by Dr. Stephen 
Mackenzie, in 1887, f° r tne British Medical Association Col- 
lective Investigation Committee. Of four hundred and 
thirty-nine cases reported by this committee thirty-four per 
cent, occurred between the ages of five and ten Years, forty- 

*The name chorea, Greek x°P eia , can be traced back to the dancing mania of the 
middle ages. During a severe outbreak of this psychic disturbance in Strasburg, in 
the early part of the. fifteenth century, the chief magistrate of that city ordered those 
affected with this dancing mania to repair to the chapel of St. Vitus, in Zabern, a small 
village not far from Strasburg. The name, St. Vitus's Dance, is the only point of affin- 
ity between the dancing mania of old and the typical chorea of the present day. 



IIO THE NERVOUS DISEASES OF CHILDREN. 

three per cent, between the ages of ten and fifteen years, 
and sixteen per cent, between the ages of fifteen and twenty 
years. The largest number of attacks were found to occur 
in the thirteenth year of life. Some cases, however, oc- 
curred very much earlier than this average percentage 
would indicate. I have seen several cases of genuine chorea 
in children under one year of age, and many more in 
children up to the age of three years. That chorea is 
also occasionally congenital must be admitted ; Sinkler re- 
fers to a case of this description due to fright of the mother 
during pregnancy. That there is hereditary predisposition 
to chorea is also evident, for the disease is developed more 
readily in children of choreic mothers and also in those 
whose parents are afflicted with epilepsy and migraine. 

All authors are agreed as to the greater liability of the 
female sex. Within the last three years I have seen 184 
cases of chorea, in which 136 were females and 48 males. 
Sinkler among 328 cases reports 232 females and 96 males. 
Gowers, who has tabulated the largest number of cases, finds 
but 365 boys among 1,000 cases. 

Dr. Weir Mitchell has studied the relation of races to 
the development of chorea, and claims that the negro race 
is almost exempt. I have no means of saying whether this 
is approximately true, but from my own experience I can 
assert that it is by no means rare among that race, but that 
among them it is if anything more frequent in boys than 
in girls. The disease is very common in Hebrews, as are 
man) 7 other neuroses. 

Climate seems to exercise but very little influence upon 
the development of the disease proper, or upon the causa- 
tion of the individual attack ; it occurs quite as freely in 
cold countries as in warm, in northern as in southern lati- 
tudes, but there is an undoubted seasonal influence. The 
great majority of attacks occur in the spring. Drs. Mitch- 
ell and Lewis have made an elaborate research on this point, 
and claim that it is not so much variation in temperature 
or in humidity that causes the attacks, but that there is a 
decided correspondence between the number of attacks of 
chorea and the number of rainy and cloudy days ; and then 
again between the attacks of chorea and the number of storm- 



CHOREA. Ill 

centres that pass over Philadelphia. Too much reliance 
should not be placed upon these statements until confirmed 
by similar researches in other cities. Putnam could not 
trace the same influences in Boston, and Gowers cannot 
accept the conclusions of Lewis for the conditions under 
which chorea occurs in England. These factors, if power- 
ful at all, are of more importance in bringing about a recur- 
rence of attacks that in giving the first impetus to the dis- 
ease. 

The causes which lead most directly to the development 
of St. Vitus's Dance are (in the order of their importance) 
fright, various acute diseases, such as articular rheuma- 
tism, scarlatina, and cardiac disease which so often ac- 
companies the acute affections just mentioned. Gowers is 
inclined to regard fright as " the only immediate cause that 
can be traced with any frequency." It was the direct cause 
of chorea in 56 of my 184 cases. As a rule, the first symp- 
toms of chorea appear within a few days of the fright ; in 
some cases within a few hours, and even within the first 
hour. I had under observation for a long time a child that 
lived near the Brooklyn Theatre at the time it was destroyed 
by fire ; the child was startled by the sight of the flames, 
and within a few hours began to twitch, and soon developed 
a severe attack of chorea which lasted for months. In one 
case the sight of a street brawl, in another the sight of a dead 
body, was directly the cause of the choreic attack. In chil- 
dren who have once had chorea very trivial occurrences 
are apt to bring about a recurrence. I have known a slight 
overstrain at. school, the unexpected report of a pistol, a 
severe thunderstorm, or a scolding by a parent to be suf- 
ficient to bring on an attack. 

The relation of acute rheumatism to the development of 
chorea has always been a matter of dispute. Some claim 
an absolute causal relation between the two, others insist 
that it is nothing more than a coincidence if one disease 
follows upon the other. Statistics upon this point are not so 
satisfactory as they might be, for cases have been reported 
in which it was stated that acute rheumatism has occurred, 
but it is not clearly made out that the rheumatism actually 
preceded the onset of the chorea. Sec reported the occur- 



112 THE NERVOUS DISEASES OF CHILDREN. 

rence of acute rheumatism in about one-fourth of his cases, 
and in these figures he is supported by the conclusions of the 
British Medical Investigation Committee and by Gowers.* 
It is a curious fact the rheumatism does not seem to pre- 
cede chorea nearly so frequently in earlier years as it does 
in those cases which occur between the ages of ten and fif- 
teen years. From this we must infer that accidental coin- 
cidence plays a very much greater role than many are will- 
ing to concede. This must unquestionably be the case with 
the statistics furnished by Dr. Hamilton, who found twenty 
per cent, of all school children choreic or affected with some 
similar disorder. 1 could find a satisfactory history of the 
chorea coming on after rheumatism in only 20 of 184 cases.f 
Hirt, in his recent text-book on Nervous Diseases, thinks that 
there is a common toxic agent which, if it affects the cor- 
tex, will produce choreic movements ; if it affects the joints 
chiefly, will give rise to acute rheumatism. This may be a 
rather hasty conclusion, but there is enough truth in it to 
say that the development of chorea is more probable in per- 
sons who have had rheumatism than in those who have not. 
The majority of recent writers, including Herringham, 
Mackenzie, Bouchard, Gowers, Osier, and others insist that 
an intimate relation exists between chorea and rheumatism ; 
but the question arises whether heart disease, which is so 
frequently associated with rheumatism, may not be in part 
responsible for the development of chorea. Statistics, in 
order to be satisfactory, should be collected very much 
more carefully than they have been hitherto, in order to 
prove in how few, or in how many, cases of chorea the first 
attack has been preceded by rheumatism or cardiac disease. 
We can readily understand that fright should be a more 
powerful agent for evil in cases in which cardiac disease 
has preceded the existence of chorea. I have found satis- 
factory evidence % of cardiac disease preceding the devel- 

* Starr has tabulated 2,476 cases (by various authors) ; in 662 (twenty-six per cent.) 
there was a history of preceding rheumatism ; in 502 there was cardiac disease. 

t In his monograph (which appeared while this chapter was going through the press) 
Osier states that there was a history of rheumatism in 15.8 per cent, of cases which he 
had examined carefully. 

+ The mere existence of a murmur has been taken by many as evidence of cardiac 
disease ; in the fewest reports is there any accurate diagnosis of the cardiac condition. 



CHOREA. I I 3 

opment of chorea in only 20 of 184 cases. The bearing that 
heart disease may have upon the pathology of chorea we 
shall have occasion to refer to later on. 

In this treatise we need not consider the chorea of preg- 
nancy, except to record the fact that it occurs, as a rule, only 
in women who have had attacks of chorea in early life. 

There has been much talk about reflex chorea, as about 
the reflex origin of many other neuroses, but he who sees 
with only half an eye will soon convince himself that these 
reflex theories are but a poor makeshift. Of all the cases 
of chorea that I have seen, I have found but very few that I 
could consider due to any peripheral exciting cause. I have 
convinced myself that in a few cases the presence of intes- 
tinal parasites was the cause of a transitory chorea, which 
disappeared as soon as the parasites were removed, but I 
am not convinced that nasal or ocular trouble, of which so 
much has been made of late, ever leads to true chorea. If 
these troubles prove an inconvenience to the child, some 
choreiform habits may for a time be established, but in 
such cases the cardinal symptoms of St. Vitus's Dance are 
wanting. 

There is a curious relation between epilepsy and chorea. 
Gowers refers to epilepsy developing from chorea, and I 
have seen a case in a woman of forty, and another in a child 
of ten years, in which severe chorea set in after the cessa- 
tion of epileptic attacks. 

Symptoms. — Involuntary and irregular movements of 
any muscle or group of muscles of the body constitute the 
chief symptom of chorea. The muscles of the hands and 
fingers, and of the face and tongue are most often affected, 
but the leg and trunk muscles are at times involved. These 
movements are aggravated by volitional effort either of the 
muscles affected or of some other group of muscles. Thus 
the choreic movements of the hands will often become very 
much more intense if the child is told to stand absolutely 
still, or if while one hand is being examined it is asked to 
grasp something with the other hand. If the patient at- 
tempt to keep the affected part absolutely quiet he may 
succeed in doing- so for a few seconds, but after that the 
movements will become more intense. 
s 



114 THE NERVOUS DISEASES OF CHILDREN. 

The choreic movements may affect only one extremity ; 
they may involve one-half of the body (hemichorea), or they 
may be generalized. In 184 cases, 35 were cases of right 
hemichorea; 32 of left hemichorea; and in 117 cases the 
choreic movements were general. 

As a rule the choreic movements are so evident that no 
special examination is needed. As the child enters the con- 
sulting-room the most superficial inspection is sufficient for 
a diagnosis ; but in other cases, particularly during the early 
stages of the disease, the choreic movements are discovered 
only upon careful examination. If there is any doubt what- 
ever about the condition, I ask the child to place its hand 
quietly upon my own, or between my two hands ; the irreg- 
ular choreic movements will, if present, be easily seen or 
felt. The true nature of the trouble, which may have ap- 
peared to be nothing more than "a slight nervousness," may 
thus be detected. If any further corroboration is needed, an 
examination of the tongue, as a rule, reveals the true char- 
acter of the disease, for there are very few cases of chorea 
in which if the movements of the extremities are ever so 
slight, the tongue does not exhibit very marked choreic 
twitching. These tongue movements are slow, coarse, 
sometimes rhythmical. In advanced cases, if the tongue 
is protruded the mouth is opened much more widely than 
necessary, the eyelids and eyebrows are raised in the same 
effort, and then through a choreic movement of the raas- 
seters the tongue may be caught between the teeth. These 
movements combined give rise to what, in a former article, 
I called the " facies " of chorea. 

The movements of the choreic patient are not only 
irregular but are often awkward to the extreme. This is 
clearly shown in the attempt to open or button the cloth- 
ing, in raising a glass of water to the lips, or in attempting 
to hold the pen in writing. This awkwardness often in- 
duces great irritability on the part of the child ; but how- 
ever annoying the movements may be, it is only in a very 
small proportion of the cases that they lead to a condition 
of exhaustion. A few years ago I had occasion to observe 
a little girl, six years of age, the child of healthy and intel- 
ligent parents. It had passed successfully through one 



CHOREA. 115 

attack of chorea , and in the second attack, coming on after 
a fright, the movements were extreme, and sleep was so 
poor that within a few weeks the child died from exhaus- 
tion. A weakened but not diseased heart unquestionably 
assisted in bringing about this early fatal termination. 
It is a fortunate circumstance that in almost all these cases 
the movements cease during sleep, and that the child is 
thus able to recover partially from the exhaustion caused 
by the movements during the day. 

Some weakness of the muscles is frequently associated 
with choreic movements. The term paralytic chorea has 
been proposed for those cases in which there is marked 
paralysis, but as there is more or less weakness in the 
majority of the cases, and often more awkwardness than 
weakness, there does not seem to me to be sufficient excuse 
for the introduction of this term. 

Speech is frequently involved. This is in the nature of 
a dysarthria rather than an aphasia, the choreic movements 
of the tongue and laryngeal muscles making speech dif- 
ficult and often unintelligible. In some cases there is a 
little awkwardness of articulation, in others hasty articu- 
lation leading to the repetition of words, and in some a 
peculiar condition of speech which is in part due to diffi- 
culties of articulation, and in part to choreic movements 
of the respiratory muscles necessitating rapid breathing. 
Deglutition may be difficult, the tongue is frequently bitten, 
and from the awkwardness in the use of the knife and fork, 
and in passing food to the mouth, the little patient is much 
annoyed and is an ungainly sight for others while at his 
meals. 

Laryngeal chorea, pure and simple, occurs somewhat 
rarely, and consists of choreiform movements of the muscles 
controlling the vocal cord. The result is a peculiar ex- 
piratory noise like a bark, which is repeated at short in- 
tervals. These cases are often mistaken for cases of hys- 
terical bark; but the general restlessness, the age of the 
patient, the choreic movements of the tongue and fingers, 
should leave little doubt regarding the diagnosis. I re- 
member a little girl, aged ten, who began to bark after a 
sudden fright ; her case had been diagnosticated as hysteria 



Il6 THE NERVOUS DISEASES OF CHILDREN. 

by several eminent physicians, but there was no element of 
true hysteria in the case. She recovered promptly under 
the usual rest treatment. There is little doubt in my own 
mind that this represents the rarest form of chorea. 

The electrical reactions are sometimes slightly altered 
in cases of chorea. Rosenthal, Benedict, and others have 
found an increased response to the faradic and galvanic 
currents on the part of the muscles and nerves of the af- 
fected side. Some have even asserted that the reaction of 
degeneration with qualitative galvanic changes occurs in 
some instances, but I am inclined to doubt the correctness 
of this statement. I have never found a similar condition 
although I have frequently examined patients with this end 
in view. If such electrical changes were present I should 
suspect the presence of multiple neuritis, as this has been 
known to occur together with chorea ; just as I might sus- 
pect this same complication in cases of marked sensory dis- 
turbances, for the rule is that in uncomplicated chorea sen- 
sation remains undisturbed. 

Mental disturbance has been frequently referred to by 
many writers as a complication of chorea. It is surely 
not a very frequent occurrence, except that in the cases 
of chronic chorea (probably a different disease) the ten- 
dency to dementia is very marked. The impression I 
recorded a few years ago seems to me to represent the 
truth of the matter : " The mental calibre of children who 
develop chorea is rather above than below par. Children 
who by means of a better mental development stand head 
of the class, who work for prizes and earn them, children 
who are under constant mental strain, and about whom 
parents and teachers make much ado, are the ones most apt 
to be attacked by chorea." In some instances a violent 
mania is developed early {chorea insaniens), but it is much 
rarer to find this sequence of events than to observe cases 
of acute mania, particularly among young girls, in whom 
the movements of the extremities and of the tongue are 
typically choreic. Irritability of temper is perhaps the 
most frequent mental condition associated with chorea ; but 
this is natural enough if we consider the very annoying 
movements and the difficulty the child sometimes expe- 



CHOREA. 117 

riences in making itself understood. At times, instead of a 
condition of mania, a condition of apathy and depression is 
present in patients afflicted with chorea ; but I am inclined 
to think that this is only true of patients who inherit a pre- 
disposition to mental disease. 

The temperature has been studied in chorea. In mild 
cases it is normal throughout the entire course of the 
disease. In severe cases it may be raised a degree or two, 
but any greater elevation is undoubtedly due to some other 
condition. 

Complications. — By far the most frequent complica- 
tions are rheumatism and heart disease. Rheumatism, if 
present, is discovered easily enough both by the fever and 
by the painful swellings ; but it is well to remember that the 
acute rheumatism of children is often a very much vaguer 
disorder than the acute rheumatism of the adult. If pain 
is much complained of in any case of chorea the joints 
should be examined carefully. Heart disease is the com- 
plication most to be feared. The heart should therefore 
be examined frequently and carefully. Mitral regurgita- 
tion is by far the most frequent form of cardiac disturb- 
ance. In the statistics of the British Investigation Com- 
mittee there were 116 cases of mitral disease and only 6 
of aortic disease. Gowers found but two instances of aortic 
regurgitation among 252 cases of chorea. Sinkler found 
cardiac murmur in 82 .of 279 cases, but he does not decide 
how many were due to organic cardiac disease. It may 
often be difficult to determine this question, but if a patient 
whose heart was normal develops a murmur while under 
observation the probability of organic lesion is very great ; 
yet since anaemia is very frequent in cases of chorea, we 
must allow, in judging cardiac conditions, for the possibilitv 
of haemic murmurs and slight dilatation of the heart. 
Brown and J. K. Mitchell have described patients covered 
with subcutaneous nodules. These have a more direct re- 
lation to the rheumatic fever than to the chorea.* 

An excess of urea and of phosphates has been found in 
the urine of choreic patients. It is questionable at best 

* Osier is of the same opinion ; he considers this condition a great rarity in this 
country. 



Il8 THE NERVOUS DISEASES OF CHILDREN. 

whether they are not in some way the result of the inces- 
sant restless movements. Convulsive attacks are referred 
to as a complication by several authors. These are not of 
a typical epileptic character, but appear to be half-choreic 
and half-spasmodic movements. Mitchell and Burr have 
recently reported a case of this sort. The cases in which 
epilepsy is associated with chorea are more than likely cases 
of organic brain lesion, in which both the hemichorea and 
the epilepsy are symptoms of one and the same process in 
the cortex. 

Duration. — It is difficult to give any accurate informa- 
tion with regard to the duration of chorea, as the disease 
can hardly be said to be ended if upon the slightest prov- 
ocation another attack sets in. A single attack may last 
from a few weeks to many months. The average duration 
is generally considered to be about ten weeks. In my own 
cases the duration of attacks varied between four and twelve 
weeks. Two and three attacks are much more common 
than a single attack. I found among 104 cases which were 
analyzed for this purpose that 50 cases had one attack; 17 
cases had three attacks ; 26 cases had two attacks ; 7 cases 
had four attacks ; 3 cases had five attacks, and 1 case had 
eight attacks. Notwithstanding this tendency to relapses 
the disease is an eminently curable one. It is only in a few 
cases that the disease becomes chronic, as in a patient of 
Meldner, who developed chorea in early life and remained 
choreic until his death, at the age of sixty-six years. 

The interval between the relapses is also subject to great 
variation. In a few cases the relapse may set in after sev- 
eral weeks ; in others after several years ; and in the case 
of chorea of pregnancy we often find that ten years or more 
have elapsed since the preceding attack. The female sex, 
for reasons too evident to mention, is more prone to re- 
lapses than the male sex. The second and third attack is 
generally supposed to be milder than the first, but there 
are exceptions to this rule, for the very worst cases of 
chorea that I have ever seen have been in patients Avho 
were passing through second and third attacks. Later 
attacks, as a rule, simulate the earlier ones. If the first 
attack has been a hemichorea it is very probable that 



CHOREA. 119 

later attacks will be of the same character. The severity 
of development, with the exceptions just mentioned, is very 
much as in the first or in the earlier attacks. 

Diagnosis. — The diagnosis of chorea rests entirely upon 
the character of the movements. These are, as a rule, un- 
mistakable, and are so typical that when they occur in con- 
nection with other diseases we speak of such movements as 
" choreic " or " choreiform." In practice the question is 
most frequently raised whether a child is suffering merely 
from general nervousness or from typical chorea. It has 
been my habit to decide this point not merely upon the 
character of the movements, though it would be safe enough 
to do this, but chiefly upon the presence of other symptoms, 
which I consider of still greater diagnostic importance. I 
refer particularly to the characteristic movements of the 
tongue, and to what I have previously alluded to as the 
" facies " of chorea. In rare instances a child may be able 
to imitate the choreic movements of others and thus simu- 
late true chorea, but if it be mere simulation the attempt 
will not be a prolonged one nor will it be successful. Hys- 
terical chorea can be distinguished very readily from the 
chorea of Sydenham by the more rhythmical character of 
the movements, by the peculiarity of the onset, by the 
longer free intervals between the attacks of twitching, by 
the longer duration of the disease, and by the presence of 
other stigmata of hysteria. 

It is not generally appreciated that the choreiform 
movements associated with infantile cerebral palsies are 
apt to be mistaken for true chorea. This post-hcmiplcgic 
chorea is very similar to the ordinary form, but it is more 
strictly unilateral ; it is more persistent, and it is invariablv 
associated with other symptoms which prove the previous 
existence of paralysis. The difficulties of diagnosis are in- 
creased by the fact that in every case of severe chorea 
there is more or less weakness of the affected members, but 
in such cases I would advise examination for the existence 
of contractures and for increase of the reflexes, symp- 
toms which are characteristic of preceding paralvsis, even 
though there be little actual weakness at the time. Rigid- 
ity and increased reflexes, moreover, are never present in 



120 THE NERVOUS DISEASES OF CHILDREN. 

cases of uncomplicated functional chorea. As this post- 
hemiplegic chorea is as much the expression of organic 
lesion of the brain as is post-hemiplegic epilepsy, it is nat- 
ural that the choreic symptoms should continue as long as 
the cerebral lesion which has given rise to them continues 
in force. In a collection of cases of infantile cerebral pal- 
sies I have found this post-hemiplegic chorea present in 
about six per cent., from which it is evident that choreic 
movements are not nearly so frequently found in conjunc- 
tion with these cerebral diseases as athetoid movements 
are. I have been consulted a number of times for persistent 
chorea, and in several such cases it has been my experience 
that the family physician has overlooked a preceding hemi- 
plegic attack which could have been made out readily 
enough if attention had been paid to the existing contract- 
ures and to the increase of the reflexes. I would urge that 
in every case of chorea a careful examination be made for 
other evidences of organic brain trouble. 

Confounding chorea and epileptiform convulsions is 
scarcely conceivable, for the convulsive movements of epi- 
lepsy come on at rarer intervals, there is generally some 
momentary loss of consciousness, and there are other symp- 
toms pointing to epilepsy. In a previous publication I re- 
ferred to the child of a colleague who would make sudden 
and very quick twitchings of an arm and of a leg. If these 
twitchings occurred while the child was walking or running 
across the room it would stand still, evidently surprised by 
these movements. It was natural to think of petit mal, 
but the frequency of the movements, the character of the 
twitchings, and the general choreic behavior of the child 
helped me to exclude petit mal and to recognize the case 
as one of true chorea. The diagnosis was corroborated by 
the very prompt result of antichoreic treatment. 

Morbid Anatomy and Pathology. — In considering 
this part of the subject we meet with very much the same 
difficulties which we encountered with regard to epilepsy, 
and the resemblance between the two is also a close one in 
this respect, that we not only have a general functional dis- 
ease, which in the one case we call epilepsy and in the other 
chorea ; but, like epilepsy, chorea is also frequently enough 



CHOREA. 



21 



the expression of actual cerebral disease. It is natural 
therefore to infer that ordinary chorea must be due to dis- 
turbances similar to those which we find in cases of organic 
lesion. Almost every conceivable change in brain structure 
has been at one time or another held responsible for the de- 
velopment of chorea. See collected 84 cases of chorea on 




Fig. 45.— Dilatation of Blood-vessels in the White Matter of the Convolutions of a very 
Chronic and Severe Case of Chorea. (Dana.) 

which a post-mortem examination had been made. In 16 
no changes were found in the central nervous system, in 32 
there were lesions in the brain and in the nerve-centres ; in 
the remainder there was congestion of the serous mem- 
branes. Ogle, Pye-Smith, and others refer to a hypersemia 
of the brain and cord. As long ago as 1868 Steiner report- 
ed upon a careful examination of three cases of chorea. He 
found cerebro-spinal anaemia and some connective-tissue 
proliferation in the upper part of the spinal cord ; conse- 



122 THE NERVOUS DISEASES OF CHILDREN. 

quently he considered chorea to be the result of spinal irri- 
tation. Meynert and Elischer found hyaline degeneration 
in the nerve-cells of the central ganglia. The latter author 
also found changes in the vessels of the central ganglia as 
well as extravasation of blood into the connective tissue of 
the brain, and also numerous emboli in the smallest vessels 
of the cortex. He described peculiar corpuscles — highly 
refractile bodies — but Wollenberg has found them in the 
brains of non-chore^c patients. Flechsig has found hyaline 
changes in the anterior divisions of the lenticular nucleus. 
More recently Dana has observed not only a general hyper- 
emia of the brain, but a degeneration in the walls of the 
blood-vessels, in the white substance of the brain, and con- 
siderable perivascular exudation Avith an accumulation of 
leucocytes. Many others, chief among them Hughlings 
Jackson, have insisted upon the embolic origin of chorea, a 
theory that would be plausible enough since Dickenson 
has found that in 17 of 22 fatal cases endocarditis was 
associated with the chorea ; and yet this theory will not 
explain that large number of cases in which there is no 
involvement of the heart. Furthermore, an examination of 
the brain in fatal cases of chorea by competent observers 
has failed to reveal the presence of emboli. This view of 
the relation between capillary embolism of the brain and 
chorea was suggested by Angel Money, who noticed after 
injections of a fluid into the carotids of animals movements 
closely resembling those of chorea, and this condition was 
found after death to be associated with capillary embol- 
ism of the brain and cord. 

Lockhart-Clarke found changes in the nerve-elements 
and connective tissue in the spinal cord ; Garrod speaks of 
an overgrowth of connective tissue in the nerve centres, 
and thus we might go on quoting as many different find- 
ings as there are authors who have Avritten upon this sub- 
ject. One of the latest contributions to this subject is by 
Anton, who found a lesion or old scar in the outermost 
division of the lenticular nucleus. As the same lesion was 
present in both halves of the brain, and the chorea was 
also a symmetrical one, the author is inclined to attribute 
the choreic disease to these lesions. By way of contrast 



CHOREA. 



123 






this same author reports the case of a man, sixty-five years 
of age, in whom spontaneous and associated movements 
were entirely wanting in the left half of the body. In the 
brain of this man the thalamus was very considerably dis- 
eased, and was supposed to be the cause of the defective 
movements. I need not discuss the author's theory at- 
tributing the excessive movements to the disease of the 
lenticular nucleus, and the defective movements to the 
thalamus. Other authors, basing their conclusions upon a 
number of autopsies, have attributed choreic movements 
to disease of the thalamus. 

In a recent article on rabies Golgi refers incidentally to his studies on 
cortical changes in chorea, which were published in 1874, and have been 
quoted since by v. Ziemssen 
and others. As a matter of 
historic interest the annexed 
figure is reproduced, showing 
the changes in the nerve- 
cells of the cerebellum ; Gol- 
gi also reports that the gan- 
glion cells of the cortex, and 
the cells of Purkinje, in the 
cerebellum, were calcified. It 
is doubtful, surely, whether 
there is any causal relation 
between these changes and 
others pointing to a chronic 
interstitial encephalitis, and 
the disease proper. More- 
over, Golgi's patient died at 
the age of thirty-two years, and 
in him chorea was associated 
with chronic mental disease. 

Up to the present time the results of bacteriological research are not very 
promising. Berkeley found the staphylococcus pyogenes aureus in cultures 
from the blood of a fatal case of chorea. 

Dana has published the history and autopsy of a case of chronic chorea. 
The patient was thirty-four years of age at time of death ; he had his first 
attack of chorea at fourteen, and repeated attacks after that. The post- 
mortem findings included a chronic leptomeningitis of the convexity of the 
brain, hyaline bodies in the brain cortex, slight meningitis of the upper part 
of spinal cord, and slight meningo-encephalitis. Diplococci were found in 
the proliferating tissue between the meninges and the brain. 

The case is of unusual interest, showing that choreic symptoms may be 




Fig. 46. — Changes in Purkinje's Cells in Chorea ; 
Varicose Swelling of the Nerve-processes. (Golgi. ) 



124 THE NERVOUS DISEASES OF CHILDREN. 

associated with a wide-spread affection ; but the true pathology of chorea can- 
not be made out in any case of fourteen years' standing, nor can such a case 
be relied upon to prove the " germ-theory of chorea." * 

The only just inference from the preceding account is 
that the accurate pathology and morbid anatomy of chorea 
are still unknown. Of the changes that have been reported 
bv various writers, many, if not most of them, are secondary 
and not primary. All that we can claim at present is that 
there is considerable change in the gray matter of the 
central nervous system ; that the entire motor tract may be 
involved, but that the changes occur more frequently in the 
cortex than in other parts of the brain. These choreiform 
movements are often associated with gross lesions in the 
cortex ; they are for this reason more common in the child 
than in the adult, and a lesion anywhere in the brain so 
situated that it cuts off cortical impulses may give rise to 
chorea. The occasional development of mental symptoms, 
the association of chorea and epilepsy, the one following 
upon the cessation of the other, are the symptoms which 
not only indicate a cerebral but also a cortical origin. 
With more recent methods of examination, such as those 
described by Golgi and Cajal, we may be able to make out 
the permanent changes in the nerve-elements of the brains 
of choreic persons, but even such changes may be second- 
ary to alteration of the blood-supply ; in fact the tendency 
of the present day appears to be to regard chorea as due 
primarily to vascular changes, and such vascular changes 
may be the result of infection. 

Prognosis. — Complete recovery is the rule in the ma- 
jority of cases of chorea. The prognosis may be a little 
doubtful in regard to the recurrence of the disease and the 
duration of an attack. A child that has once had chorea 
has acquired a distinct predisposition to the disease, and 
often an occurrence which would leave no impression upon 
a healthy child's nervous system is sufficient to bring about 
a relapse of the disease. Later attacks are generally as 

* Osier refers to the bacteriological researches of Pianese (Naples, 1893). Animals 
inoculated with a culture of bacillus taken from a choreic patient died " with muscular 
twitchings and convulsions," and the same bacillus could be obtained in pure cultures 
from the central nervous system. But it is doubtful whether these animals had chorea. 



CHOREA. 125 

mild as the first, and there is no special reason to fear an 
unfavorable issue in later attacks unless severe complica- 
tions set in. Under such circumstances not the chorea, but 
the complicating rheumatism, or endocarditis, is the actual 
source of danger. Death occurred in only two per cent, of 
the cases collected by the British Medical Investigation 
Committee, and Sinkler states that in Philadelphia, in 
seventy-four years, there have been but sixty-four deaths 
from chorea. This latter statement does not mean very 
much, as the majority of the cases of chorea ending fatally 
would be reported as cases of one of the complicating con- 
ditions. 

There is no way of predicting positively the duration of 
a choreic attack. The milder an attack at the beginning 
the more likely it is to run a short course, whereas the 
severer forms are apt to be much more chronic, but severe 
cases under proper treatment will yield much more quickly 
than mild cases that are handled improperly. Under com- 
petent medical care the first attack may be recovered from 
in a period varying from four to ten weeks unless serious 
complications arise. A few get well more quickly, but they 
are the exceptions rather than the rule. 

Treatment. — A severe case of chorea puts the skill of 
the attending physician to a severe test, while in a mild 
case the less medical interference the better for the child. 
Increased experience, both in private and in dispensary 
practice, prompts me to urge the simple plan of treatment 
which I outlined a few years ago. 

The most important factor in the treatment of chorea is 
rest, absolute rest, often to the exclusion of all other thera- 
peutic measures. Take a choreic child that has been ac- 
customed to roam about at will and put it to bed ; it will be 
a little restless for the first few days, but it soon quiets down 
and shows the great advantage of a thorough rest in bed. 
There is difficulty occasionally in carrying out this plan, for 
mothers and nurses are only too likely to be disheartened 
by the first show of resistance on the part of the child and 
its unwillingness to remain quiet ; but with a few exceptions 
this restless opposition on the part of the child disappears 
within a few days, and the little patient feels very much 



126 THE NERVOUS DISEASES OE CHILDREN. 

happier in bed than out. After a few days of enforced rest 
a decided improvement is noticeable. In the milder forms 
all movements cease, and in the severer forms the child is 
no longer troubled by the annoying jactations of the limbs. 
According to the severity of the case rest should mean en- 
tire rest in bed day and night. If the disease is taking a 
favorable course, after a week or two the patient may be 
taken out of bed. for half an hour, an hour, or two hours, 
and then returned again for the remainder of the day. I 
have met only very few cases in which it seemed impossible 
to carry out this treatment ; but I am firmly convinced that 
it was never the fault of the little patient but always the 
fault of incompetent and unintelligent relatives. If the dis- 
ease has assumed a mild form we can endeavor to keep the 
child quiet without keeping it in bed the entire day ; a few 
hours' rest will be better than none. It will also be of bene- 
fit to the child to forbid its taking any violent exercise, such 
as running, riding, dancing, or bicycling. 

I am so convinced of the value of this rest treatment in 
chorea that I have made it a rule, even in dispensary prac- 
tice, to insist upon this point of rest ; we go to the extent 
of preferring the mothers to report to us about the child 
rather than to have the child taken out of bed and brought 
to us at short intervals. 

Next in importance to rest is a nutritious and easily di- 
gestible diet. Milk and rest will do more for most cases of 
chorea than any other two measures. The nutritious diet 
will have a peculiarly good influence upon the many cases 
that are associated with profound anaemia. 

The monotony of this special form of rest-cure can be 
varied with advantage by the use of lukewarm baths. Im- 
mersion into a cold bath, or the wet pack, with subsequent 
friction, cannot be recommended. It is better to place the 
child in lukewarm water, then reduce the temperature by 
adding cold water, and with this water, that is growing 
colder and colder, to sponge the spine thoroughly in order 
to get the effect of the dripping water upon the skin. After 
the bath the patient should be kept quiet and wrapped up 
warmly. In every case proper hygienic and dietary meas- 
ures are of far more importance than medicinal treatment, 



CHOREA. 127 

and yet we are bound to consider the various drugs that 
have been suggested for the cure of this disease. 

Among these arsenic holds the first place. Its praises 
have been warmly sung by some, while others have decried 
it as but little better than any other drug that might be 
substituted for it. Only a few years ago Dr. Seguin, in his 
remarkably lucid lectures on the various forms of functional 
neuroses, placed arsenic first and rest second in the treat- 
ment of chorea. This order I think should be reversed, as 
I have yet to see the first case of chorea that got well more 
quickly with arsenic than without, as long as it was getting 
the benefit of rest. Dr. Seguin insists that physicians, 
almost without exception, give nearly useless doses of ar- 
senic. He regards eighteen to twenty-seven drops of Fow- 
ler's solution after each meal as the really efficacious dose. 
In my own experience very few children will tolerate these 
large doses, which should at all times be given in some alka- 
line water within an hour after meals. I have always con- 
tented myself with smaller doses, varying from four to 
twelve drops three times a day, and if the cases resisted 
treatment I prefer abandoning arsenic rather than pushing 
it to the extreme which Seguin recommends. In cases of 
excessive restlessness I have been in the habit of prescrib- 
ing the arsenic, together with the elixir of the bromide of 
sodium, or if the sleep is disturbed I give the evening dose 
only in this way and administer the arsenic alone during 
the day ; the free use of chloral and bromides is to be con- 
demned. If one must use any drug in the cases of chorea, 
arsenic is to be preferred ; but it cannot be expected to per- 
form miracles, and we must not regard it in any sense as a 
specific therapeutic agent. 

Many other remedies have been proposed, all have been 
tried, and almost all have been abandoned. For some time 
the tincture of cimicifuga was in great favor. From fifteen to 
thirty drops, three times daily, may be administered in those 
cases in which arsenic is not well borne h\ the stomach. I 
have seen no good reason to resort to the use of hypodermic 
injections of arsenic, as recommended by Eulenburg and 
Hammond ; Weir Mitchell some time ago proposed the 
use of salicylates; Simon and Legroux suggested the use 



128 THE NERVOUS DISEASES OF CHILDREN. 

of antipyrin ; and the oxides and sulphate of zinc have long 
been in popular favor in the treatment of this disease ; but 
no one, I think, would venture conscientiously to recom- 
mend any of these drugs as a specific against chorea. The 
preparations of iron and of arsenic fulfil this role better 
than any other drugs, for they at least help to tone up the 
general system. Hirt recommends the use of morphine. 
If this drug were as effective as it is claimed to be, its use 
in young children is not to be encouraged. In the earlier 
stages of chorea it is essential for patients to obtain sleep. 
According to the age of the child chloral in five, ten, or fif- 
teen-grain doses per rectum may be given. If it is neces- 
sary to substitute another drug I would suggest the use of 
chloralamid, of sulphonal, or trional (ten to fifteen grains 
each), and if there is a great deal of mental excitement I 
should favor the hypodermatic use of the hydrobromate of 
hyoscine (one two-hundredths to one one-hundredth grain). 

In the majority of cases a heart tonic will be necessary. 
Digitalis in drop doses of the fluid extract or the tincture 
of strophanthus should be given in cases of heart weakness 
or feeble pulse. Blaud's pills, the sirup of the iodide of 
iron, various preparations of cod-liver oil, good stimulating 
wines, all these will be called for in some cases of chorea, 
but whatever else one may be induced to give the only 
matter of importance is that absolute rest shall be enforced. 

Erb has advised the use of electricity. A weak gal- 
vanic current may be employed safely enough and may be 
applied to the nape of the neck, and over the motor areas ; 
in which case, if given late at night, the current will help 
to bring on normal sleep. A moderate stabile current of 
from fifteen to twenty cells (about ten milliamperes) ap- 
plied to the spine will help to allay restless movements of 
the body. I am opposed to the use of a strong current to 
the head, as it may do much more harm than good, and 
also to the use of the faradic current to the head or spine, 
which would act as a direct irritant rather than as a seda- 
tive. Massage may be given in some cases in which the 
general nutrition is at a very low ebb and in Avhich the 
circulation is poor. 

A special caution is necessary as regards the question 



CHOREA. 129 

of attendance at school. Every choreic child, however 
mild its attack may be, should be kept from school both 
for its own sake and for the sake of the other pupils who 
might imitate the disease. I have sometimes allowed my- 
self to be persuaded to permit a child with a mild form of 
chorea to continue at school ; in almost every instance I 
have had reason to regret it, for nothing is better calculated 
to bring out severe chorea than the competitive spirit that 
obtains in most schools. Periods of examination are fraught 
with greatest danger to those children who have had attacks 
in earlier life. The atmosphere of the school-room seems 
to have a depressing influence upon such children, and 
among the wealthier classes far better progress can be made 
in the ordinary studies if the child is instructed at home 
than if it is taught at school. It is necessary for the phy- 
sician to take a firm stand on this question or else his treat- 
ment of the case will be thoroughly unsatisfactory. 



BIBLIOGRAPHY. 

Anton: Neurolog. Centralbl., 1893. 

Benedikt : Nervenpathologie und Elektrotherapie, p. 256. 

Berkeley : Johns Hopkins Hosp. Reports, 1891, vol. ii., No. 6. 

Brown : Journal of Nervous and Ment. Dis., 1891. 

Dana : Brain, vol. xiii. , April 1890. (Cases with autopsy collected.) On 
the Microbic Origin of Chorea, Am. J. of Med. Science, January, 1894. 

Elischer : Virch. Arch., vols. lxi. and lxiii. 

Erb : Elektrotherapie, p. 587. 

Golgi : Ueber die Pathologische Histologic der Rabies Experimentalis, Berl. 
kl. Wochenschr., No. 14, 1894, and Rivist. Clin., iv., p. 361, 1874. 

Koch : Ueutsch Arch. f. Kl. Med., 1887, p. 40. 

Knapp : Review of Infectious Origin of Chorea, Boston Med. and Surg. 
Journal, January 25, 1894. 

J. K. Mitchell : Univ. Med. Magazine, vol. i. 

Naunyn : Schmidt's Jahrb., January 15, 1889. 

Osier : Chorea and Choreiform Affections. Philadelphia, 1894. 

Roger: Arch. Gen de Medecine, December, 1866, and January, 1867. 

Rosenthal : Klinik der Nerven Krankheiten, 1875. 

Rothschild : Arch. f. Kinderheilkunde, vol. xvi.. p. S^. 1893. 

Sachs : Article Chorea, Keating's Cyclopaedia, and Hare's System of The- 
rapeutics. 

Sahli : Deutsches Arch. f. Kl. Med., vol. Ii. ; on rheumatism. 

See : Memoires de^l'Acad. de Med., tome xv., 1850. 

Q 



130 THE NERVOUS DISEASES OF CHILDREN. 

Sinkler : In Pepper's System of Medicine. 

Soltmann : Article on Chorea, in Gerhardt's Handbook, contains Litera- 
ture up to 1880. 

Starr : Article Chorea, in Am. Text-Book of Children's Diseases. 

Virchow: Hirsch Jahresbericht, 1889, Bacteriological Investigations. 

Wollenberg : Zur Pathologie d. Chorea Minor, Arch. f. Psych., vol. xxiii., 
p. 167. 

V. Ziemssen : Article Chorea, in Ziemssen's Cyclopaedia (full Literature), 
1877. 



CHAPTER VI. 

CHOREIFORM DISEASES. 

HEREDITARY OR HUNTINGTON'S CHOREA. 

This disease is, on the whole, extremely rare. In view 
of its hereditary character we must consider it in connec- 
tion with the diseases of children, although it generally 
appears about the age of thirty or forty. The disease was 
first observed by Huntington, a Long Island physician, and 
it has since been designated by his name. It appears, how- 
ever, that another New York physician, Waters, described 
the disease in a letter to Dunglison. For a long time the 
disease was little known beyond America. Of later years 
it has been observed and discussed by a number of English, 
German, and French authors. The disease runs in families 
and is spread from one community to another in connection 
with the migration of the afflicted families, so that now, ac- 
cording to Gray, there are a number of communities in 
America in which the disease is prevalent ; but, as he further 
states, there is great secrecy maintained with regard to it, 
as the affliction is looked upon as a distinct stigma resting 
upon the family. 

The disease affects several members of the same genera- 
tion, but may skip as many as it selects. The descendants 
of healthy members of a family enjoy immunity from the 
disease as a rule. In King's case, which I quote on the au- 
thority of Gray, the great-grandfather was choreic. He had 
ten children, but only four were afflicted with this disease; 
they also had children who were choreic. One of these four 
had nine children, eight of whom were healthy, but the 
ninth was choreic. The ninth had live children, of whom 
four were choreic. Of these four, three had no children, 
but the fourth had a chorea whilst he was still young and 



132 THE NERVOUS DISEASES OF CHILDREN. 

was cured of it, when again at thirty-five he passed gradu- 
ally into the typical Huntington's chorea. 

Symptoms. — The disease begins, as a rule, between the 
ages of thirty and forty years, though at least a dozen or 
more cases are on record which have begun before the age 
of twenty. Males and females seem to be afflicted with 
the disease in equal numbers. The chief symptom is a 
motor disturbance, which for lack of a better name is termed 
choreic, yet it is very different from the twitchings of 
chorea minor. The muscular movements of Huntington's 
disease are coarse and grimacing, and may be distributed 
over a large area of the body. In Sydenham's chorea the 
affection is more strictly localized. In the former disease 
grimaces and all sorts of extravagant posturing are much 
more pronounced than those which we observe in the 
ordinary chorea of children. The difference in degree of 
muscular activity is so very great that one naturally doubts 
the connection between the two diseases. As a rule, the 
movements are slight at the start, affecting the face and 
upper extremities only. In the course of years they in- 
crease in intensity, and become widely distributed over the 
entire body, until in the end every single muscle or group 
of muscles may be involved. 

The muscular disturbance is in part subject to the will, 
and patients afflicted with this disease can, if they make a 
serious effort, inhibit the twitchings very much better, and 
for a much longer period, that those suffering from ordinary 
chorea can. The motor power is, as a rule, not diminished, 
but on account of the irregularity of muscular actions it is 
extremely uncomfortable for the patient to walk about, and 
he easily becomes an object of pity or of ridicule. 

There are no sensory disturbances, and the reflexes, 
while they may vary much, are not morbidly exaggerated 
or diminished. By far the most characteristic symptom of 
Huntington's chorea is the association with it of a progres- 
sive dementia. The first sign of a mental change may be 
a simple depression which is deepened by a knowledge 
of the hereditary and incurable character of the disease. 
Under the circumstances the occurrence of suicide is not 
unnatural. The mental condition at the start varies a little ; 



HEREDITARY OR HUNTINGTON'S CHOREA. 1 33 

the patient may be either irritable or apathetic. In the 
course of time the deterioration of all the mental faculties 
is very marked and a typical dementia is developed. Speech 
is, as a rule, thick and indistinct, sometimes nasal and con- 
fused. Unless some intercurrent disease puts an end to 
life these patients may linger on for many years, may be- 
come absolutely bedridden, a burden to themselves and a 
torment to their families. 

The course of the disease is a very chronic one, and 
unfortunately does not tend to shorten human life. Sev- 
eral of those who have exhibited the first symptoms of dis- 
ease at the age of thirty have lived to sixty and seventy 
years. Whether cases beginning very early in life live 
as long, cannot be distinctly stated, but there is nothing 
improbable in supposing that they do. 

Etiology. — Among the causes of this disease none is 
more potent than heredity. It is distinctly a family dis- 
ease, and unlike other such diseases it does not seem to 
skip a generation. Persons with this disease can generate 
healthy children whose descendants may not be afflicted 
with this trouble. To all appearances an exciting cause is 
needed to develop the disease. In many of the cases the 
first symptoms appear upon severe emotional excitement 
or after some acute disease. In this respect the resem- 
blance between the hereditary chorea and chorea minor 
is very striking. Rheumatism does not, however, play the 
part in the hereditary form that it does in ordinary chorea. 

Differential Diagnosis. — The strong factor of hered- 
ity and the appearance at a relatively advanced age are 
sufficient to distinguish these cases from ordinary chorea. 
There is some danger, however, of confusing these cases 
with the post-hemiplegic chorea that is developed early in 
life, and this danger is all the greater if the onset of the 
paralytic symptoms is uncertain, or if the paralysis has 
disappeared and the chorea remains. From Friedreich's 
ataxia the disease can be easily differentiated bv the fact 
that in the former malady the disturbance of motion is 
truly ataxic and not choreic, and the reflexes are totally 
absent, whereas in Huntington's disease the reflexes are 
not markedly altered. In Friedreich's disease there is also 



134 THE NERVOUS DISEASES OF CHILDREN. 

ataxic gait, made worse by closing of the eyes, whereas in 
Huntington's disease nothing of the sort is observed. 
Some forms of hysterical chorea might be confounded with 
this disease, particularly if on inquiry the statement is elic- 
ited that a similar affliction has been observed in other 
members of the family, and it should not be forgotten that 
the hereditary trouble may be of an hysterical order. The 
examination of the patient and the general hysterical tem- 
perament, the fact that the choreic movements come on in 
the nature of attacks, and the determination of other hys- 
terical symptoms will help to distinguish one disease from 
the other. 

Pathological Anatomy. — The morbid changes in 
Huntington's chorea have not yet been definitely made 
out. Innumerable changes have been reported by various 
authors. These include pachymeningitis, hsematoma, tu- 
mors of the dura, general atheroma, atrophy of the motor 
convolutions, increased fluid in the ventricles, foci of soft- 
ening in various parts, even in the ganglia. The most 
frequent states are pachymeningitis and changes in the 
cortical tissue. In a case of Charcot's the meninges were 
adherent in certain places, and the cortical substance was 
evidently sclerotic. But these varying conditions are 
largely secondary processes and do not in any way explain 
the true pathology of the disease. Dr. Osier summarizes 
the changes in a series of sections from the brain and cord 
which he examined as follows : " The arteries were thick- 
ened, and, in places, show hyaline degeneration, and in the 
smaller arterioles the fatty changes were very marked in 
the fresh specimens from the cortex. The perivascular 
lymph-spaces were large, and contained leucocytes. The 
ganglion cells, in many sections, showed very slight changes, 
not more than are often seen in chronic cases associated 
with atrophy of the convolutions. There was the common 
vacuolation, and many cells seemed laden with pigment. 
The increase in the connective-tissue elements was more 
evident to the touch and upon section than microscopically. 
Sections of the pons and medulla showed no foci of disease. 
Beyond the thickening of the arteries and the shrinkage 
of the cells of the anterior cornua — probably an artificial 



HEREDITARY CHOREA WITHOUT DEMENTIA. 135 

change — the sections of the cord showed no important 
lesions."* For the present we can say nothing more than 
that the disease is, in all probability, due to some chronic 
cerebral process, the exact nature of which will have to be 
determined by further post-mortem examinations with the 
aid of improved methods of staining. 

Prognosis. — The prognosis of Huntington's chorea is 
grave as regards the cure of the trouble, and in view of the 
marked dementia which is associated with almost every case. 
Unfortunately, however, it is not a fatal disease, and patients 
so afflicted may live on to a very old age. 

Treatment. — As for the treatment of these cases the 
same principle should be observed as in ordinary chorea — 
rest, freedom from care, and excitement, separation from 
family, and change of climate, and possibly a course of 
arsenic treatment may be of some benefit. If movements 
are excessive and the sleep of the patient is unsatisfactory, 
the exhibition of hyoscine, in doses of one one-hundredth of 
a grain, or of trional or chloralamid, in ten- to twenty-grain 
doses, once or twice a day, is worthy of a trial. But, in the 
nature of things, every form of treatment will be simply 
palliative. 

HEREDITARY CHOREA WITHOUT DEMENTIA. 

There is another form of chorea of which a slight mention should be made 
in this connection. The disease has a distinct hereditary tendency, and is 
either transmitted direct from parent to child or from a more remote ances- 
try. The disease appears early, beginning as a rule at about the age of 
puberty and continuing during life. It is characterized by distinct choreic 
movements of the hands and tongue and the facial muscles. The move- 
ments may become so marked that they interfere with every voluntary effort, 
such as riding, sewing, buttoning of clothes, and the like. The lower ex- 
tremities do not become affected, and the general health of the patient is not 
much impaired, except that the annoyance of the disease may bring on a 
despondent feeling ; and in one case that has come under my notice distinct 
melancholy has occurred. It is distinguished from Huntington's chorea 
above all by the entire absence of symptoms pointing to dementia. The 
persons afflicted with this form remain bright throughout life and are able to 
attend to their ordinary affairs. It is furthermore to be distinguished from 
chronic chorea from the fact that there is no distinct history of an acute 
attack after which the chorea has been developed ; that the choreic move- 

* Greppin claims that the disease is due to a morbid process (not unlike an encepha- 
litis), which is developed gradually and in various parts of the brain. 



136 THE NERVOUS DISEASES OF CHILDREN. 

ments have never been as wide-spread as they are in such cases of chorea 
minor as develop into chronic chorea. They generally begin a little later in 
life, too, than the acute chorea minor does, and there is no distinct history of 
individual attacks. If the disease has once been established it continues 
without any marked remission, but also without any rapidly progressive 
changes. Some confusion might also arise with the post-paralytic chorea, 
but under such circumstances an examination into the past history of the 
patient, and for the evidences of persistent paralysis, will help to clear up the 
diagnosis. 

The prognosis is entirely favorable as regards life, but less can be said as 
regards the cure of the disease. I have placed such patients under the com- 
plete rest-cure, and have for a time obtained distinct improvement in the 
choreic movements. General tonic measures should be employed, and the 
proper feeding of the patients carefully looked after. 

HABIT CHOREA. 

It is well Known that children are subject to " tricks " of movements. 
These include simple or co-ordinated movements of various muscles, gen- 
erally of the muscles of the face, of the eyes, of the shoulder, and even of the 
thighs. These " habit spasms " or " tics " may resemble chorea ; often the 
jerkings are so violent in character as to suggest " epileptoid " rather than 
" choreic " disease. 

It is not always an easy matter to determine how the " trick " was en- 
gendered ; in some instances a true chorea has been the starting-point, the 
children keeping up some form of twitching movement after the St. Vitus's 
dance has disappeared. Through imitation, habit chorea may be developed 
in children who have watched others with chorea minor. 

A habit chorea developed in early years is often continued throughout 
life. Among professional men (artists, literary men, and even physicians) 
such " tricks " are not uncommon, and the doubt arises whether serious ef- 
fort has been made to dispel the habit. Among the commoner forms of 
" habit chorea " are blinking, facial contortions, sniffing, shrugging of the 
shoulders, or some trick in speech or gesture. A friend of mine, now a well- 
known astronomer, has since boyhood never answered a question without 
first saying, Hm ? ah ? eh ? In former years this utterance was accompanied 
by an exaggerated raising of the eyebrows, as if to intensify the interrogation. 

Such habits cannot be condemned sufficiently in children. Parents 
should discipline children severely in order to rid them of the habit. 

If there is any peripheral condition (such as nasal obstruction or eye- 
strain) which helps to keep up the habit, the condition should be treated 
carefully. It is important also to make certain that the " habit spasm " is 
not true chorea. This can be done by examining for other symptoms of St. 
Vitus's dance. The general condition of children with some form of habit 
spasm may require treatment ; many of them are anaemic or scrofulous. 

Complex Co-ordixated Movemexts (Complex Ticsl— In children 
as well as in adults, complex movements of a definite character are repeated 



COMPLEX CO-ORDINATED MOVEMENTS. 1 37 

at intervals, or may be continuous. They bear only a very slight resemblance 
to chorea ; but, as a matter of convenience, they may be discussed in connec- 
tion with "habit chorea." Some of them, the gyrospasms, for instance, 
occur in very young infants ; others occur in older children and are frequently 
associated with slight mental derangement. Some authors have included 
them under the heading of Imperative Movements, and have described them 
as head-shaking, head-rotating, head-banging, head-nodding, etc. These 
movements are often associated with nystagmus and with defective mental de- 
velopment, thus indicating that in some instances they may be due to organic 
disease of the brain. These peculiar disorders have been studied carefully 
by Henoch, Hadden, Gee, Peterson, and Mills, and have been referred to by 
Osier and others. 

Gyrospasms of the Head is a term applied by Peterson to peculiar ro- 
tary movements of the head in children, associated at times with strabismus 
or nystagmus. Two of Peterson's five cases were observed by him in my 
clinic. Both these cases were in young infants, eight and nine months of age 
respectively ; in one the movements came on after a fall ; in the other there 
was no history of traumatism. The chief points of Case II., as described by 
Peterson, are as follows : The child was nine months Old when examined. 
Since the age of four months he had a rotary movement of the head. When 
the child was quiet, the head kept oscillating from right to left and from left 
to right at the rate of eighty to one hundred oscillations per minute. The 
movement ceased at times, particularly if its attention was riveted upon 
some object. There was lateral nystagmus of the right eye, also ceasing 
for minutes at a time. The patient had measles two months previously ; no 
convulsions ; no fall ; labor had been entirely normal. The general health 
was good. 

These gyrospasms, which are evidently of a piece with head-nodding, 
head-jerking, etc., are scarcely to be confounded with epilepsia nutans. If 
developed in later years, they may constitute a habit, but if the movements 
begin in early infancy and are associated with nystagmus, strabismus, or 
idiocy, one need not hesitate to ascribe them to cerebral disease. The re- 
gion of the cranial-nerve nuclei would be the most probable seat of the 
trouble ; and the lesion must be supposed to be irritating in character. 

The chief etiological factors appear to be rickets, intestinal irritation, and 
dentition. The prognosis is good on the whole, except when these move- 
ments are associated with idiocy. A mild course of bromides has proved 
satisfactory in the majority of cases. Mills recommends, in addition, the use 
of two or three minims of tincture of belladonna, or one minim of the fluid 
extract of conium. 

Imperative movements associated with arithmomania (repeating every- 
thing a definite number of times) are clearly the result of mental derange- 
ment, and do not seem to me properly to belong to this group of cases 



Several other motor neuroses bearing a more or less 
close resemblance to chorea minor have been described in 



138 THE NERVOUS DISEASES OF CHILDREN. 

connection with the chief disease. While the cases may 
have a superficial resemblance to St. Vitus's dance they 
are, as a rule, of a much more explosive character. They 
occur in families with a neurotic heredity, and represent, 
on the whole, a more serious disturbance of the central 
nervous system. It is not an easy task properly to classify 
all these conditions. There has been much confusion in 
the discussion of these subjects, and no two authors entirely 
agree in the designation of these disorders. As a majority 
of them come on later in life, or at least are fully developed 
in later years, we need not in this book treat them in a very 
exhaustive manner. 

CHOREA ELECTRICA. 

It is certain that two distinct forms have been described under this 
term. Henoch includes under it a form of choreic disturbance which re- 
sembles in part what is commonly designated as habit spasm, but some of 
his cases are more distinctly allied to myoclonia or paramyoclonia multiplex. 
In the latest edition of his book Henoch does not insist on the old designa- 
tion, yet many authors have followed him and are continually quoting his 
cases under the name of chorea electrica. 

The term electric chorea should, however, be restricted entirely to a very 
rare disease that occurs chiefly in the northern part of Italy. I have had 
opportunity to see one case of this description in Italy, and one in an Ital- 
ian woman who came to my clinic. The condition was first described by 
Dubini, in 1846, and to avoid confusion it would be as well to speak of it as 
Dubini's disease. The disease is apt to occur in boys and girls as well as in 
men and women of advanced years. It has been supposed to be due to some 
infectious agent, but the rarity of the disease, even in those districts in which 
it does occur, would seem to militate against this view. 

The chief symptom of the disease is a series of violent spasmodic move- 
ments, affecting particularly the muscles of the neck and head, as well as of 
the extremities. Some of the cases begin with movements in an arm, which 
spread to the leg of the same side, and finally involve the opposite half of 
the body, and also the trunk, neck, and head. After some months the 
choreic members of the body become paralyzed, there is wasting of the mus- 
cles and a loss of faradic irritability. Epileptiform seizures occur, and these 
may either be partial or general. In the course of a year or more the pa- 
tient may become entirely paralyzed, and, as in the case I saw, the patient 
was confined to his bed, unable to move a limb, while the severest choreic 
jactation continued in the muscles of the neck. The majority of these cases 
end fatally within a few weeks or months. During the course of the disease 
the patient suffers a great deal from pain, has slight elevation of tempera- 
ture, but, as a rule, does not have loss of consciousness. 



MALADIE DES TICS CONVULSIFS. 1 39 

The disease is so rare that sufficient pathological examinations have not 
been made to warrant definite statements with regard to its nature. It is 
probable that it is a form of cerebro-spinal disease, as is indicated by the con- 
vulsions on the one hand and the paralysis with atrophy on the other. 



MALADIE DES TICS CONVULSIFS. 

This disease, also known as Gilles de la Tourette's disease, rep- 
resents another form of motor neurosis, not unlike some of the symptoms 
presented by electric chorea. The chief symptoms of this condition are con- 
vulsive twitchings of the facial muscles and other regular systematic move- 
ments, explosive conditions of speech known as echolalia and coprolalia, and 
lastly, imperative conceptions and impulses. The disease, as a rule, appears 
in children between the ages of seven and fifteen years. It is more apt to 
occur in those predisposed by inheritance to neurotic troubles than in others. 
In many instances there is a very distinct history of heredity, the same dis- 
ease occurring in succeeding generations. 

The first symptoms that appear are sudden and explosive twitchings 
of the muscles of the face or of the eyes. The mouth may be twisted, and 
all sorts of grimaces may constitute the early symptoms. In a case which 
I have had under observation, the boy would begin by making grimaces 
and then turn about quickly as though he was snapping at some one. 
These movements were performed in regular succession. Movements of the 
sterno-cleido mastoid, and of the trapezius are often repeated so systemat- 
ically that they seem to be purposive. The child may at the same time begin 
to spit or to blubber, and the entire combination of symptoms suggest the 
possibility of simulation or intention, particularly if jumping or leaping move- 
ments are associated with the other symptoms. Whatever the symptoms 
may be that have been developed in a given case they are persisted in with 
remarkable regularity, and can be distinguished from intentional movements 
by the fact that the patient is evidently surprised by the suddenness and vio- 
lence of the movements. Smacking, hissing sounds are sometimes heard, 
but none of the symptoms is more characteristic than the habit of repeating 
words or sounds that are heard (echolalia), or the involuntary sudden explo- 
sive utterance of foul language (coprolalia). A little patient of mine uttered 
the worst curses I ever heard, which she had evidently picked up on the 
street, but would regret the utterance the very next moment after they had 
passed her lips, and would contritely declare that she was entirely irrespon- 
sible for the same. 

The symptoms frequently continue for years, and while the intelligence of 
the person so afflicted does not show any deficiency only a few are able to 
become masters over these explosive seizures. In some an attempt to con- 
quer the disease produces great restlessness and general excitement. In 
others the symptoms become manifestly worse under the effort to control 
them, and it is advisable under such conditions to divert the attention of the 
patient from his symptoms as much as possible. 

Imperative conceptions are frequently associated with the motor symp- 



140 THE NERVOUS DISEASES 0E CHILDREN. 

toms, and usually seem to represent a psychic explosion, the equivalent of the 
physical symptoms. Some of the children feel compelled to utter words in a 
definite sequence, to pronounce certain letters in a peculiar way ; rolling the 
" r " and making a hissing " s " are particularly frequent. In others impera- 
tive actions, such as occur in connection with neurasthenic disorders, are quite 
common. They are compelled to do their sums over and over again, to re- 
trace their steps, to pick up everything they see lying on the street or in the 
room, and are compelled to be in a state of continued activity in consequence 
of these imperative impulses. 

The course of the disease is extremely chronic ; by some it is considered 
to be absolutely incurable. But this is too extreme a view, since some of the 
cases get well. 

The prognosis should, however, be studied carefully in each case, as there 
is no telling in advance whether the patient will respond favorably or not to 
the treatment. The resemblance between this disease and other choreiform 
neuroses is at times so close a one that a differential diagnosis becomes ex- 
tremely difficult. It can hardly be confounded with ordinary chorea since 
the movements are much more systematic and more intermittent than they 
are in St. Vitus 's dance. Moreover, echolalia and coprolalia never occur in 
ordinary chorea. The difficulties of speech in chorea are the very reverse of 
the explosive speech in this maladie des tics. Hysteria and hysterical chorea 
may bear a superficial resemblance to this disease ; but, on the one hand, the 
stigmata of hysteria are generally wanting, and in hysteria there is no such 
constant repetition of more or less convulsive movements as there is in this 
disease. There is, no doubt, a close resemblance between the maladie des 
tics and the jumpers and many other forms of disease which are differently 
designated according to the peculiar variety of muscular action. The one 
point, however, which the maladie des tics has in common with other similar 
conditions is its occurrence in neurotic families. The distinction between 
maladie des tics and para-myoclonia will be evident after a discussion of the 
latter trouble. 

Treatment. — The most important factor in the treatment of these pa- 
tients is their complete isolation from all other children and from their usual 
surroundings. They should be subjected to a moderately severe discipline by 
a competent nurse or parent, and should be taught self-control as far as that 
may be possible. The mere seclusion from other persons generally serves to 
lessen the excitement and the number of explosions. If the disease has led 
to a condition of exhaustion from loss of sleep or from inanition it is well to 
secure quiet during a considerable part of the entire day. This can be done 
best by the administration of chloral or of small doses of morphine. I can- 
not see the wisdom of using chloroform, as has been suggested by some. If 
the motor restlessness is extreme, hypodermic injections of hydrobromate of 
hyoscine, in doses of one one-hundredth grain, carefully administered, may 
prove of some benefit ; the ordinary nerve-tonics, such as arsenic and quinine, 
will do little good, and it is best not to waste much time in administering 
these drugs. I am confident that if any good is to be accomplished we must 
depend entirely upon isolation, proper feeding, and the use of tonic hydro- 



THOM SEN'S DISEASE. 14 1 

therapeutic procedures. Regular gymnastic or calisthenic exercises should 
be tried and may be of advantage. 



thomsen's disease (myotonia congenita). 

In connection with the various forms of disordered movements which 
have been considered in this chapter, we may discuss in a very brief way the 
disease known by the name of the physician who gave the first thorough 
description of it as it occurred in his own family. Those who object to 
nomenclature of this sort will prefer the term Myotonia congenita, but as 
Thomsen was also a subject of the disease an exception may well be made 
in his case. 

The disease is unusually rare. In this country very few cases have been 
described, and the only one which the author has had an opportunity of see- 
ing was the one presented to the New York Neurological Society, in 1886, by 
G. W. Jacoby. The best accounts of the disease are those furnished by 
Thomsen himself, a description of it in Leyden's work on " Spinal Cord 
Diseases," and the monograph of Erb, who summarized the chief points of 
twenty-two cases, and added a full account of peculiar electrical changes in 
this disease, for which he proposed the term " Myotonic Reaction." 

The chief symptoms of the disease are a rigidity of the muscles (myoto- 
nic contraction), which is developed whenever an attempt is made to use 
a muscle or muscles after a period of rest. If the first difficulty has been 
overcome, the action of the muscles may be entirely normal for some period 
of time. This muscular rigidity is most noticeable in the attempt to rise 
after a person has been quietly seated for some time ; in the attempt to use 
the hands in lifting, grasping, or writing, and at times considerable difficulty 
is experienced in the movements of the tongue and of all muscles concerned 
in articulation. The impression created by a patient grasping the physician's 
hand and then not being able to let go, or by his falling to the ground and 
remaining absolutely rigid until the spasm is relaxed, is one not easily for- 
gotten. The spasms are generally limited to a few groups of muscles, but in 
some cases the entire muscular system, with the possible exception of the 
ocular and respiratory muscles, is involved. The rigidity of the muscles is in- 
creased under the influence of emotional excitement and under the influence 
of cold and damp weather. Under alcoholic stimulants the rigidity is said to 
lessen, and prolonged periods of rest undoubtedly have a favorable influence 
upon the disease. 

The disease occurs in families, and very often affects several members of 
the same family. In Thomsen's family, five generations have been affected 
by this same condition. There seems to be no distinction as regards sex, 
and the disease may come on at any period of life. A number have been re- 
corded during the first decade of life, but the disease is apt to reach its maxi- 
mum during the period of greatest muscular development, and the symptoms 
are, therefore, pronounced between the ages of fifteen and twenty-five. In 
Jacoby's case the patient, who was twenty-four years of age at the time of 



142 THE NERVOUS DISEASES OF CHILDREN. 

examination, stated distinctly that he could not, as a boy, take part in the 
outdoor games of his comrades on account of the stiffness of his muscles. 
He attempted to play upon the organ, but found that his hands were clumsy. 
He was not able to whistle, and even in chewing his food the muscles be- 
came stiff and rigid, simulating a condition of trismus. In this case there 
was distinct rigidity of the muscles of the eyes. 

The entire muscular system is, as a rule, well developed ; there is often an 
increase of normal volume. According to Thomsen, the more the muscles 
are used the less the spasm becomes, and he advises a very active life as pos- 
sibly the only hope for an improvement in the disease. 

There are a few objective symptoms which render the diagnosis of the 
disease easy enough. The mechanical excitability of the muscles is mark- 
edly increased, a single tap of the hammer on a muscle produces a slow, tonic 
contraction of the fibres, but the contraction is not relaxed for some period 
of time. In contradistinction to the symptoms of tetany it may be stated 
that the nerves do not show any increased mechanical excitability. There 
is, furthermore, a very remarkable change in the behavior of the muscles un- 
der electrical stimulation. Erb has proposed to speak of this as " myotonic 
reaction." The faradic excitability of the nerves is not changed, but on the 
use of a strong current the muscles innervated by the nerve will be forcibly 
contracted and remain contracted for some period of time after the current is 
broken. The direct faradic excitability of the muscles is very much in- 
creased, mild currents being sufficient to produce contractions of long dura- 
tion. The galvanic excitability of the nerves is surely not increased — possi- 
bly diminished — but the same muscular phenomenon can be observed on 
galvanic stimulation of the nerve as in the case of faradic excitation. The 
direct galvanic excitability of the muscles is increased ; the anodal contrac- 
tion is, as a rule, greater than the kathodal contraction ; opening contrac- 
tions either with the anode or kathode cannot be readily obtained. The con- 
tractions are sluggish, and are continued for some time after the elec- 
trical stimulation ceases. But the most characteristic symptom of this 
myotonic reaction is a peculiar, rhythmical, wave-like contraction which pro- 
ceeds from the kathode toward the anode. This phenomenon can best be 
observed with the use of strong currents of at least 20 or 25 milliamperes, 
and if the negative pole is placed over the tendinous end of a muscle. If 
the kathode is placed in the palm of the hand, the anode on the shoulder, a 
wave-like contraction will appear and gradually work its way from the mus- 
cles near the wrist to those of the shoulder. 

These are the chief symptoms of the disease. A few complications have 
been observed in some of the cases, among which we may specially mention 
increased mental irritability and hypochondriasis, both of which are not un- 
natural in view of the annoyance which the disease causes the sufferer. 
Epilepsy and migraine have also been recorded in connection with Thorn- 
sen's disease, but as both these diseases are to a great degree hereditary the 
association maybe simply a coincidence. 

The pathology of the disease is still obscure, and up to the present time 
no post-mortem examination has been made. The muscular tissue has been 



PARAMYOCLONUS MULTIPLEX. 1 43 

excised from the living body and examined. The changes noted were an 
enormous hypertrophy of the fibres, with considerable increase of the nuclei in 
the sarcolemma, and an increase of the interstitial connective tissue. In one 
case examined by Erb, there was a striking vacuolization of the individual 
muscular fibre. These histological findings do not enable us as yet to explain 
the character of the disease. All that we can say is that there must be some 
congenital condition which causes a hyper-excitability of the ganglion cells of 
the cord. Whether the condition causing this hyper-excitability is in the 
muscular tissue itself, in the nerves, or in the spinal cord, cannot be definitely 
stated. Thomsen considers defective cortical innervation to be the prime 
cause, but cerebral changes alone could not account for all the symptoms ; 
these must be ascribed to disease (primary or secondary) of the muscles or 
to disease of the cord. 

The disease can be mistaken only for tetany, and possibly for chronic mus- 
cular dystrophies ; but as Erb suggested, several years ago, a tap with the 
percussion-hammer and a few closure contractions with kathode and anode 
upon certain muscles will suffice for a diagnosis of Thomsen's disease. 

The prognosis is favorable as regards life, unfavorable in regard to a cure 
of the disease. Much can unquestionably be done by a continued exercise of 
the will and by regular gymnastic exercise. No further treatment of the dis- 
ease is indicated. 

CONGENITAL PARAMYOTONIA. 

Eulenburg * has described a somewhat similar affection, which he terms 
" Congenital Paramyotonia." This also is a distinctly family disease which 
may be traced through six generations, the disease becoming manifest im- 
mediately after birth. The chief feature of these cases is a tonic spasm last- 
ing from a quarter of an hour to several hours, and coining on chiefly after 
exposure to cold. There is distinct weakness associated with rigidity ; the 
facial muscles may be involved as well as the orbicularis palpebrarum and 
the orbicularis oris, making speech impossible during the continuance of the 
contraction. The disease is differentiated from Thomsen's disease by the 
absence of increased mechanical excitability and by the absence of anything 
resembling a myotonic reaction. Eulenburg states that there is a distinct 
tendency to kathodal and anodal tetanus. 



PARAMYOCLONUS MULTIPLEX (MYOCLONIA). 

A short reference is necessary in this connection to the above disease, 
which bears a resemblance to electrical chorea and to tic convulsif. By 
some it has been regarded as a form of hysteria, but this is not warranted. 
for cases have been observed in which there were none of the characteristic 
signs of hysteria. It is best to restrict the term paramyoclonus multiplex 
to the disease as it was originally described by Friedreich. The chief symp- 

* Neurologisches Centralblatt, June 15, 1SS6. 



144 THE NERVOUS DISEASES OF CHILDREN. 

torn of the condition is a rapidly repeated clonic spasm, occurring in attacks 
and affecting individual muscles or groups or muscles, and as a rule symme- 
trical muscles are involved. The muscles of the extremities are more fre- 
quently affected than those of the other parts ; the face muscles are gener- 
ally exempt, and in this respect the disease differs markedly from other forms 
of choreic and clonic spasm. These clonic convulsions are not severe 
enough to cause actual movements of the extremities, but in a few cases sud- 
den contractions of the diaphragm have been accompanied by hiccough and 
by peculiar respiratory sounds, and in the Case described by Starr the move- 
ments were strong enough to produce jactitation of the head and of the 
trunk. In contrast to some of the other diseases described in this chapter, 
the myotatic irritability is increased only a little or not at all. Electrical ex- 
citability is not altered. 

The disease may come on at any period of life between the age of pu- 
berty and fifty years. It is rare in young children, and the short account of it 
here is simply given for the purpose of showing its close relationship to the 
various forms of choreic disorders. Heredity is not a prominent factor in 
the cases which have been described, but it has been found in several mem- 
bers of one family. Mental or physical strain and emotional excitement are 
the chief causes. Cases closely resembling, yet not identical with paramyo- 
clonus multiplex have been described by Hammond and others, under the 
title of " Convulsive Tremor." 

The prognosis is serious as regards a cure of the condition, though some 
of the cases have been reported improved and a few entirely cured. In the 
treatment of the disease nothing more can be attempted than regular gym- 
nastic exercise, and the exhibition of mild sedatives, such as chloral and the 
bromides, in small doses, in case the spasms are excessive. A mild galvanic 
current is said by some to have exercised a favorable influence over the dis- 
ease. 



BIBLIOGRAPHY. 

HUNTINGTON'S CHOREA. 

Berkeley : Medical News, Philadelphia, 1883, vol. xlii., p. 200. 

Bourneville et Pilliet: Arch, der Neurol, 1887, 1888. 

Charcot : Progres Medic, 1878. 

Ewald : Zeitschr. f. kl. Med., vol. vii., 1884, p. 51. 

Fere: Arch, der Neurol., January, 1884. 

Greppin, L.: Archiv. fur Psychiatrie, vol. xxiv., p. 155. 

Gray : Text-book, pp. 321-338. 

Herringham : Brain, vol. xi., p. 415. 

Hoffman: Virchow's Arch., vol. cviii., 1887. 

King : New York Medical Journal, 1885. 

Marie : Progres Medic, 1886. 



CHOREIFORM DISEASES. 1 45 

Menzies : Journal of Mental Science, October, 1892, to June, 1893. 

Oppenheim and Hoppe : Arch. f. Psych., vol. xxv., p. 617. 

Osier : Journal of Nervous and Mental Diseases, February, 1893. 

Peretti : Berl. Klin. Wochenschr., 1885, No. 52. 

Phelps, R. M. : Journal of Nervous and Mental Diseases, 1892. 

Schlesinger : Zeitschr. f. Kl. Medicin, 1892. 

Zacher : Neurologisches Centralbl., January 15, 1888. 

HABIT CHOREA, GYROSPASM, ELECTRICAL CHOREA, ETC. 

Gee : St. Bartholomew's Hospital Reports, vol. xxii. 

Gowers : Diseases of Nervous System, vol. ii., p. 633 (" Habit-spasm "). 

Hadden : Lancet, June 14, 1890, and St. Thomas's Hospital Reports, 1890. 

Henoch : Lectures on Diseases of Children. 

Janowitz : These de Paris, July, 1891. 

Mills : Chapter on Imperative Movements, etc. American Text-Book of 

Diseases of Children, p. 712. 
Osier: Monograph. On Chorea, 1894. 
Peterson: Medical News, October 1, 1892. 
Weir Mitchell : Medical News, March 4, 1893. 

MALADIES DES TICS. 

Charcot : Lecons du Mardi, 1887-88. 

Dana: New York Medical Record, 1887. 

Gilles de la Tourette : Archives de Neurologie, 1885 ; Nos. 26 and 27. 

Grasset : Arch, de Neurologie, 1890. 

Guinon, G. : Revue de Medecine, 1886, i., 1887, p. 500. 

Hammond : New York Medical Record, February 27, 1892. 

Jolly : Charite Annalen, 1802, p. 740. 

thomsen's disease. 

Bernhardt: Virchow's Archiv, 1879, vol. lxxv. 

Dejerine et Sottas (Case with autopsy) : Compt. Rend, de Biol., Paris, 1893 

(not accessible to author). 
Erb : Monograph. Leipsic, 1866. 

Eulenburg and Melchert : Berliner Kl. Wochenschr., No. 38, 1885. 
Eulenburg : Neurologisches Centralbl., 1886, p. 265. 
Jacoby, G. W. : Journal of Nervous and Mental Diseases, March, 1887. 
Marie : Revue de Medecine, 1883, p. 1064. 
Seifert: Arch. f. Kl. Med., 1891, vol. xlvii., p. 127. 
Striimpell : Berliner Kl. Wochenschrift, 1881. 
Thomsen, J.: Archiv f. Psychiatric vol. vi., 1876, p. 702; also vol. xxiw, 

1892, p. 918. 
Westphal : Berliner Kl. Wochenschr., 1883. 
White: Digest, Brain, vol. ix., p. 112. 



146 THE NERVOUS DISEASES OF CHILDREN. 



PARAMYOCLONUS MULTIPLEX. 

Bechterew : Neurolog. Centralblatt, 1887, p. 181. 

Friedreich : Virchow's Archiv, vol. Ixxxvi. 

Homen : Arch, de Neurologie, 1887. 

Jolly: Charite Annalen, 1892 (On " Tics "). 

Marie : Progres Medical, 1886. 

Remak : Arch. f. Psychiatrie, vol. xv. 

Seeligmuller : Deutsche Med. Wochenschr., 1886, No. 24. 

Starr : Journal of Nervous and Mental Diseases, July, 1887. 

Unverricht : Die Myoclonic Vienna, 1891. 






CHAPTER VII. 

TETANUS. 

In view of recent bacteriological researches tetanus may 
be described as an infectious disease of the central nervous 
system, characterized by continuous tonic spasms with oc- 
casional attacks of clonic movements. The muscles first 
affected are, as a rule, those of the neck and jaw. Opistho- 
tonus and lock-jaw (trismus) are the first and most startling 
symptoms of the disease. According to its origin we dis- 
tinguish between traumatic tetanus resulting from a wound, 
and idiopathic or rheumatic tetanus if the cause is unknown, 
or if, in our ignorance, we attribute it to exposure to wet 
and cold. We must take special note of the tetanus oc- 
curring in new-born children (tetanus neonatorum), and 
may make a passing reference to puerperal tetanus. There 
is every reason to believe that traumatic injury, with the 
attendant invasion of the bacilli of tetanus, is the primary 
cause of the trouble in every instance. 

Etiology. — Tetanus is far more frequent in males than 
in females. This is particularly true of those cases in which 
the traumatic factor is evident, and is easily explained by 
the greater liability of the male sex to traumatism. The 
very fact that of 46 so-called idiopathic cases recorded by 
Gowers only 9 were in females would lead one to doubt 
the spontaneous origin of these cases. While tetanus may 
occur at any time of life, it is most frequent between the 
ages of ten and forty years. It occurs within the first few 
days of life between the fourth and eleventh days, but be- 
tween this time and the age of five years there is, practically, 
immunity. Between five and ten years the tendency to 
the disease is on the increase, and continues to increase un- 
til after the age of forty years. These statistics strengthen 



148 THE NERVOUS DISEASES OF CHILDREN. 

the impression that traumatic injury alone, plus infec- 
tion, is the only feasible explanation of the occurrence of 
the disease. 

Colored races, and particularly those living in tropical 
countries, are affected more frequently than white people. 
This has been amply proved in regard to the population of 
India and the West Indies. The filth and uncleanly habits, 
so common among these people, should be borne in mind. 
Puerperal tetanus, which is unquestionably infective in 
character, occurs very frequently still in these same coun- 
tries, but it is becoming less and less frequent in those 
countries in which modern surgical principles prevail in 
obstetrical practice. 

The previous health of the person seems to have little to 
do with the development of the disease. The most robust 
as well as the feeblest are equally liable to the infection. 
Irregular wounds, burns, scratches, and frost-bites are more 
often the cause of tetanus than the large aseptic incisions 
made by the surgeon's knife. Tetanus has been known to 
follow the extraction of a tooth, and, in the popular belief, 
lock-jaw is much feared after slight injuries to the toes 
in paring nails, etc. With greater cleanliness among the 
masses the disease will no doubt soon diminish. Tham- 
hayn states, from a large collection of cases, that tetanus 
is developed most frequently from wounds in the hand, leg, 
foot, head and neck, arm and trunk, in the order of fre- 
quency just mentioned. Injuries to the head have often 
been supposed to be peculiarly liable to produce tetanus ; 
but as such injuries are generally the result of a fall and 
contact with the earth, as in a fall from a horse, it will be 
seen that the danger of infection is greater than in many 
other instances. The same is true of tetanus following a 
splinter which has been run under the nail, and the old- 
fashioned method of stopping bleeding by putting cobwebs 
around the bleeding part may be very largely responsible 
for the tetanus. The micro-organisms of tetanus evidently 
do not require a large abraded surface, and it is question- 
able whether, even in those cases in which tetanus comes on 
after flogging, there was not sufficient abrasion of the epithe- 
lium to permit of the invasion of these minute organisms. 



TETANUS. 149 

As a rule, tetanus is developed within a period of five to 
fourteen days after the initial injury; but, in some cases, a 
few hours are sufficient for the development of the disease, 
and in cases in which the crural nerve has been enclosed 
by a ligature, full-fledged tetanus of the severest type was 
observed at once after the occurrence. The development 
of tetanus is not rare within three or four weeks after the 
injury, but beyond such a period the occurrence of tetanus 
could not well be explained. That infection alone will not 
explain all the circumstances in connection with the de- 
velopment of tetanus will be insisted on in the section on 
pathology. 

Symptoms. — The first symptoms of tetanus, if we except 
vague pains at the seat of injury, and a dull headache, is 
a gradual stiffening of the muscles of the neck and of the 
jaw until the head is markedly retracted (opisthotonus) 
and the jaws are firmly clenched (trismus). While the cer- 
vical muscles are apt to be involved at the beginning the 
dorsal and lumbar apparently escape ; but retraction of 
the head is soon followed by arching of the back. As the 
disease continues the legs become rigid, but the arms es- 
cape or are but little involved. The muscles of the face 
are often affected at an early stage of the disease. The eye- 
brows are raised, the ocular fissure becomes narrow, the 
mouth is distorted, the lips press against the teeth and the 
entire face may assume what is known as the " risus sar- 
donicus." The rigidity of the muscles is painful, and the oc- 
casional clonic exacerbations which occur tend to make the 
condition still more distressing. The difficulties of degluti- 
tion and of respiration, from spasm of the thoracic muscles 
and of the glottis, increase the agony of the patient and are 
a continuous menace to life. Epigastric pains (pains possibly 
due to spasm of the diaphragm) are frequently complained 
of. During sleep the spasm usually ceases, but no sooner 
does the patient Avake up than all the distressing symptoms 
return in full force. 

The pulse is rapid and feeble. Whether vasomotor 
spasm is the cause of this peculiarity of the pulse has not 
been sufficiently determined. The temperature varies 
considerably ; in some instances it remains normal during 



150 THE NERVOUS DISEASES OF CHILDREN. 

the entire course of the disease, in others there is a con- 
tinuous fever of from two to three degrees. H. C. Wood 
refers to the serrated tracings of the temperature chart, 
the rise coinciding with the paroxysm, and the fall of the 
line with the interval. Occasionally the temperature rises 
to io8° or even no F., and according to Wunderlich this 
high temperature continues for some hours after death, 
reaching even to 1 14 F. It was formerly supposed that the 
rise of temperature was due to excessive muscular action ; 
but cases in which muscular action is greatest are not nec- 
essarily those with highest or even high temperature. 
Gowers is inclined to consider the fever to be of nervous 
origin, which is probably correct if modified to mean that 
the toxines circulating in the system exercise a special ir- 
ritating influence upon the centres in the pons and spinal 
cord, or upon the heat areas in the cortex. Thirst is a fre- 
quent and most distressing symptom of the disease, and as it 
cannot be easily quenched, on account of the difficulty of 
deglutition, it adds much to the discomfort of the patient. 
The thirst is also increased by the very profuse perspira- 
tion which sets in and is a prominent factor in many of the 
cases. 

The urine is scanty and high-colored, of high specific 
gravity, all of which can be explained by the excessive ac- 
tion of the skin. Micturition is often irregular, possibly in 
consequence of spasm of the sphincter. The bowels are, as 
a rule, constipated. 

All the symptoms are apt to increase in intensity as the 
disease progresses. The majority of cases run to a fatal 
termination in less than a fortnight, often in four to five 
days. Death occurs, as a rule, either from failure of the 
heart or from asphyxia in consequence of spasm of the re- 
spiratory muscles. In other cases exhaustion from the con- 
tinued spasms and from the inability to take food may be 
the ultimate cause of death. Cases lasting above a fortnight 
are supposed to tend toward recovery. The spasms be- 
come slighter, the clonic spasm less frequent, and all the 
symptoms gradually recede until nothing but a slight rigid- 
ity of the muscles remains. The parts last involved are first 
released, and the spasms last longest in the muscles of the 






TETANUS. 151 

neck and jaw, the very parts which were first affected. A 
single recovery seems to provide immunity against the dis- 
ease for all time. While the evidence upon this point is 
not absolutely conclusive, the fact of immunity after the 
first attack would be in keeping with what we know about 
immunity from other infectious diseases. 

Varieties.— The majority of the cases are so much alike 
that it would be quite useless to speak of variations from 
the clinical type. An exception must be made, however, 
in favor of what Rose has termed " cephalic tetanus." This 
form develops, as a rule, after injury to the head, and partic- 
ularly to the region of the face supplied by the fifth nerve. 
The special characteristics of this form are the association 
of paralysis of the face on the same side as the injury with 
tetanic spasms of the other side. In addition to this we 
find spasms of the respiratory muscles and great difficulty 
in deglutition. The behavior of the patient is very much 
like that of one suffering from hydrophobia, hence this 
form has also been spoken of as " tetanus hydrophobicus." 
There is some doubt whether injury to the peripheral nerve 
is the cause of the facial palsy. Bernhardt and Remak 
proved conclusively that there was no reaction of degen- 
eration in the nerve or muscles, and the nerve has been 
found healthy on post-mortem examination. We must 
suppose, therefore, that the facial palsy in such cases is of 
reflex origin. Some support is given to this view by the 
fact that if recovery sets in, the tetanic spasm and the facial 
palsy disappear at about the same time. 

The only other variety worthy of special mention is the 
so-called abortive form of tetanus described by Kussmaul. 
In these cases spasm of the neck muscles and trismus are 
the only symptoms, and these gradually disappear. 

Tetanus neonatorum can hardly be considered a variety 
of tetanus, for it resembles in every way the tetanus of the 
adult, but in this book it will naturally deserve some spe- 
cial consideration. 

The tetanus of the new-born child sets in, as a rule, be- 
tween the fifth and tenth days after birth, though it may be 
delayed as long as twenty days. The first symptom noticed 
in the child is difficulty in taking the nipple or in drinking 



152 THE NERVOUS DISEASES OF CHILDREN. 

from the bottle ; with every attempt there is distinct rigid- 
ity of the masseters and of the muscles about the mouth, 
which interferes with the act of sucking and swallowing. 
Other facial muscles are apt to be in a state of contracture 
leading to a distinct deformity of the face. In some in- 
stances food can be poured into the mouth by a spoon with- 
out exciting a spasm, but in many others the mere touch of 
the spoon to the lips, or contact of the food with the lips 
and mouth, is sufficient to produce a spasm. 

In the earlier stages of the disease the child is quiet un- 
less food is given; but before long the spasms come on 
spontaneously without excitation by food. Spasm of the 
respiratory muscles soon forms a part of the clinical symp- 
toms, and periodic cyanosis is often one of the early symp- 
toms of the tetanic condition. Rigidity of the muscles of 
the neck and back, typical opisthotonus, and arched back 
soon follow, and to make the symptoms thoroughly com- 
plete the upper and lower extremities become the seat of 
muscular spasms. There may be slight remissions for a 
time, but the tetanic stage becomes more and more perma- 
nent, or, if absent when the child is quiet, will surely come 
on with the merest touch in lifting the child, in trying to 
give it an enema, in washing it, etc. The temperature, as a 
rule, remains normal ; in a few instances it reaches 102 or 
103 F. 

The disease is steadily progressive. One is often de- 
ceived by slight remissions and is flattered by the tempor- 
ary good result of sedative treatment, but is only too often 
disappointed by the reappearance of all the symptoms, with 
even greater intensity. Death may follow within a period 
varying from one and a half to eight or nine days, and re- 
sults either from mere exhaustion, due to the inability to 
take food, or from asphyxia, due to spasm of the inspiratory 
muscles. 

A few cases of tetanus neonatorum get well, but surely 
only a very few. Cases with fever seem to be invariably 
fatal. In those who get well the tetanic spasm disappears 
very slowly, and Henoch states that in some instances a 
rigidity of the muscles existed three weeks after the onset 
of the disease. 



TETANUS. 153 

The cause of this early tetanus is in every way the 
same as that of tetanus in the adult, except that the en- 
trance of the bacilli into the body occurs by way of 
the umbilical cord, the care of which has been neglected. 
In former days, and particularly in those countries in which 
obstetrical cases were managed exclusively by midwives, 
whose ideas of cleanliness were very defective, tetanus 
neonatorum was very much more frequent than it is now. 
Ritual circumcision, if performed without due regard to 
modern surgical principles, may be considered an occasional 
cause of tetanus. Bathing a child at low temperatures is 
referred to by many of the older writers as a common 
cause of tetanus. The use of a bath thermometer is said to 
have put an end to an epidemic of tetanus occurring in the 
practice of a midwife who was not able to distinguish by 
the hand alone a high temperature of water. In view of the 
modern bacillary theory of tetanus such an origin of tetanus 
would be rather difficult to explain ; but we must concede 
the possibility of other than mere toxic agents affecting and 
irritating the peripheral nerves of a newly born child. 

Pathological Anatomy and Morbid Pathology. — 
Careful search has been made by many writers for the act- 
ual lesion of tetanus, but up to the present time with few 
positive results. It has been noted that rigor mortis sets in 
with unusual promptness, some claiming that the tetanic 
spasm passes without relaxation into the post-mortem con- 
tracture. All the organs of the body have been examined ; 
no special changes have been found in them. Some writers 
have reported oedema, hypostatic pneumonia, pleural ex- 
travasation, which can be accounted for by the inter- 
ference with the circulation and respiration. The muscles 
of the body are sometimes ruptured ; some contain small 
hemorrhages, but under the microscope the fibres present 
no anomalies, though Bowman refers to occasional granular 
degeneration. It has been natural to look for changes in 
the nerve filaments in the peripheral wound which has been 
the starting-point of the tetanus. In some instances noth- 
ing abnormal has been found ; in others there has been 
neuritis, with considerable swelling of the nerve, which ex- 
tended from the periphery to the spinal cord. 



154 



THE NERVOUS DISEASES OF CHILD REX. 



In the case of tetanus neonatorum an inflammation of the 
umbilical vessels has been traced for some distance within 
the abdomen, and the peritoneal covering of the vessels has 
also been found inflamed. 

As for the central nervous system, the condition most 
frequently discovered is one of passive distention of the 
vessels, associated with minute hemorrhages : but both these 
conditions are surely secondary to the tetanic spasms and 
not in any sense the cause of them. In the spinal cord some 
investigators have reported granular disintegration of the 
gray substance, the formation of cavities containing gran- 
ular material, and changes 
also in the large ganglion 
cells, such as were found by 
Xerlich in the motor root 
of the fifth nerve. All of 
these changes are not suffi- 
ciently distinct to represent 
the actual pathological con- 
ditions of tetanus ; they are 
secondary effects, and it is 
probable that in tetanus, as 
in other of the diseases 
hitherto considered, definite 
though the symptoms may 
be, the changes are of a 
transitory character, every trace of which disappears after 
death. It would be more important, therefore, to refer to 
the morbid pathology. 

The pathology of tetanus can be readily understood in the 
light of modern researches if we regard the spasms of tet- 
anus as the result of increased irritability of the convul- 
sive centres in the brain. This increased irritability is di- 
rectly due to the influence of germs or germ products 
introduced into the system from some external injury. 
The tetanus bacillus (Nicolaier) is a rod-shaped microbe 
whose spores are attached to one end of the bacillus, which 
with its spores resembles in appearance an ordinary pin. 
This microbe is found in the soil as well as in the dust of 
dwellings. These bacilli are capable of resisting great heat, 




Fig. 47. — Tetanus Bacilli and Spores. 
(x about 1,000. ) (Kkasato.) 



TETANUS. 



155 



retaining their activity even after exposure to 175° F. for 
an hour (Kitasato, ZeitscJirift fiir Hyg., vol. vii.). Their 
spores are capable of resisting the same heat for six hours, 
but the spores are not formed if the temperature exceeds 
108° F. The bacilli thrive in an atmosphere of hydrogen 
or in vacuo. They are killed by exposure to the oxy- 
gen of the air and by a five per cent solution of carbolic 
acid. 

As products of these bacilli we have several poisons 
(tetanin, tetano-toxin, and also tox-albumhis, Brieger), which 
have been derived from pure cultures and which are ca- 
pable of exciting the disease. It is probable that the tetanus 
bacillus itself does not cause the disease, but that the poi- 
sonous substances formed in the blood by the presence of 
this bacillus are the direct cause of tetanus. It is an inter- 
esting fact that granulating surfaces do not offer a suitable 
soil for the production of these toxic substances. The 
presence of oxygen seems to prevent the penetration of the 
bacilli into the tissues underneath ; hence we can readilv 
understand why scratches and hidden injuries should be 
far more dangerous than large abraded surfaces. 

Tetanus was supposed to result from direct irritation of 
the peripheral nerves ; if so, an ascending neuritis would 
have to account for the development of the symptoms of 
tetanus. It is at present altogether impossible to deny that 
in some way or other the bacillus must have been intro- 
duced into the system even in these cases.* 

Lesions of the occiput were supposed to lead more fre- 
quently than other lesions to tetanus, but in this instance, 
too, it is more than probable that the infectious element 
cannot be disregarded. 

In no disease have bacteriological researches led to 
more satisfactory results in treatment. Tizzoni cultivated 
the virus, inoculations of which in increasing strength pro- 
duced tetanus in dogs. The blood-serum of such dogs 
destroyed the activity of the virus. He inferred the pres- 

* Since the above was written Goldscheider has given a different explanation based 
upon recent anatomical conceptions. The poison of tetanus acts upon the peripheral 
nerve-fibre. This fibre being the peripheral portion of the neuron, an increased ex- 
citability of the ganglion cell is produced, and this hyper-excitability brings about 
the tetanic contractions of the muscles with which it is connected. 



156 THE NERVOUS DISEASES OF CHILDREN. 

ence of an albuminoid body, which he called "anti-toxin/* 
with which he succeeded in arresting the disease in rats. 

The now famous researches of Behring and Kitasato 
have revealed the fact that the blood-serum of tetanic ani- 
mals produces immunity in others, and that the serum of 
the animals thus rendered immune had still more active 
anti-toxic qualities, and during the past year (1894) Behring 
has shown that the blood-serum of immunized animals not 
only produces immunity in others, but also has a distinctly 
curative effect. Tarnuffi (Centralblatt fur Barter iologie, 1892) 
reports a successful use of anti-toxin injections in the case 
of a peasant, aged seventy-four, who had developed tetanus 
as the result of a lacerated wound of the little finger of the 
right hand. One hundred and fifty grammes of the anti- 
toxine was given in this case, when on the eleventh day 
after the beginning of the injections a complete recovery 
ensued. The blood-serum from this patient did not produce 
tetanus when injected into a rat. No doubt other success- 
ful cases of this kind will soon be reported, and there is 
much to hope from this serum therapy in the case of tet- 
anus. 

Differential Diagnosis. — The diagnosis of tetanus is 
easy in the majority of cases. It is possible to mistake 
tetanus for strychnia poisoning, but in the latter the symp- 
toms never begin with distinct trismus. The symptoms 
also develop with much greater rapidity, and the severe 
pain referred to the stomach, as well as the absence of ex- 
ternal injury, would lead one to suspect the effect of strych- 
nia. 

If there is great difficulty in deglutition the symptoms 
of tetanus may resemble somewhat those of hydrophobia, 
but the entire absence of trismus, and the fact that all the 
symptoms are excited only if an attempt is made to swallow, 
and the previous history of the case, will enable one to dis- 
tinguish hydrophobia from true tetanus. 

As for mistaking tetanus for tetany, such confusion is 
scarcely possible if the mode of onset from the periphery 
inward, in cases of tetany, is kept in mind, and if we also 
remember that no such interval exists in tetanus as in the 
lesser disease. The favorable course of the disease will 



TETANUS. 157 

render still further aid, after the lapse of a few days, in dif- 
ferentiating between tetany and tetanus. 

Prognosis. — The prognosis is invariably grave. A mor- 
tality of eighty -five per cent, does not overstate the facts. 
Other things being equal, the prognosis is the more grave 
the more thoroughly a lacerated wound has been infected. 
Recovery in a case of tetanus after compound fracture 
of the limbs is very rare. The longer the interval between 
the injury and the first onset of the symptoms the better 
the prognosis, and cases which begin after ten days usually 
take a more favorable course than those which come on 
very early after the injury. If the symptoms have lasted 
more than ten days there is some reason to expect a fa- 
vorable result. On the whole it is a disease very much to 
be feared, and a guarded prognosis is quite in order until 
a very decided improvement sets in ; but possibly the recent 
discoveries of Behring may in the course of time enable us 
to be more hopeful. 

Treatment. — In the treatment of tetanus, prophylactic 
measures are by far the most important. There is no good 
excuse at the present day for the infection of any surgical 
wound, or if the wound has been received under conditions 
which make infection possible, thorough antiseptic measures 
should be at once employed, and even after the first symp- 
toms of tetanus have been observed the condition of the 
peripheral wound should be carefully examined into and 
everything should be done to prevent further contamina- 
tion and infection. 

If recent investigations are carried to a successful issue, 
the most satisfactory and the most rational treatment of 
tetanus will unquestionably be that by injections of anti- 
toxin or any of the substances which may hereafter be 
proved to possess the property of counteracting the poison 
of tetanus in the human system. It is a consummation 
most devoutly to be wished, but though we are nearing the 
goal the struggle is not yet at an end. 

In the majority of cases, and particularly in those occur- 
ring at a distance from large medical centres, from which for 
years to come these anti-toxins will have to be obtained, the 
older methods of treatment will still have to be resorted to. 



158 THE NERVOUS DISEASES OF CHILDREN. 

The general management of the patient is the first im- 
portant consideration. Absolute quiet should be secured 
by darkening the room, keeping out all visitors and noises. 
Nutritious but liquid diet should be given, and given slowly, 
so that the difficulties in deglutition may not be increased. 
If the trismus is very marked a wedge may have to be in- 
serted between the teeth to secure enough space to intro- 
duce a tube through which liquid can be taken into the 
mouth. If this cannot be done, the patient must be fed 
through the nose or else by the rectum. 

In former times it was considered wise to remove a 
peripheral scar, or even to amputate the part that included 
the seat of injury ; but according to our present conception 
the presence of tetanus bacilli in the system would prove 
that the disease was no longer a local one, and that excision 
of the lesion would not prove sufficient to bring about re- 
covery. 

Among the drugs employed in the treatment of this dis- 
ease chloroform by inhalation is unquestionably the most 
satisfactory. Woods, of Philadelphia, has recommended the 
use of nitrite of amyl, and Gowers reports that its use at 
Guy's Hospital has proved that the spasms became more in- 
tense at first but slighter afterward. Both chloroform and 
nitrite of amyl are palliative measures, but not curative in 
any true sense. Chloral hydrate has an advantage over 
the preceding drugs, inasmuch as it helps to produce sleep, 
and can be given continuously for a long period of time. 
To be effectual large doses of five to fifteen grains should be 
given at a time, and repeated several times during the day 
according to the needs of the case. 

There can be no reasonable objection to the use of opium 
and morphia in the form of suppositories, or by hypo- 
dermatic injection. Morphia may be exhibited in doses 
of one-twelfth to one-sixth grain, according to the age of 
the child, and can be given in connection with other nar- 
cotics. The use of conium and of gelsemium has been 
warmly recommended by various observers, but the results 
are not satisfactory enough to advise the general adoption 
of such measures. Curara in doses of one-fiftieth to one- 
half grain every hour has been urged by some ; but it is 



TETANUS. 



I 59 



surely a dangerous drug, and its application would be 
rather in the nature of an experiment than of safe thera- 
peusis. Continued warm baths, electricity (galvanism to 
the spine and the muscles), all these measures have been ad- 
vocated by some, but without much result. If the bromides, 
chloral, opium or morphia, and chloroform, judiciously ad- 
ministered, do not bring about a favorable result it is safer 
not to exhibit other drugs. 

In the treatment of tetanus neonatorum the same drugs 
may be employed as are recommended in tetanus of the 
adult, but the dosage should be modified in keeping with 
the age of the patient. 



BIBLIOGRAPHY. 

Behring and Kitasato : Deutsche Med. Woch., Dec. 4, 1890, and 1892, p. 

348. 
Brieger: Deutsche Med. Wochenschrift, 1887. 
Conner, P. S. : Article Tetanus, Pepper's System, vol. v. 
Frothingham : Tetanus Bacillus, American Journal of the Medical Sciences, 

May, 1894. 
Goldscheider : (Action of Tetanus Toxin, etc.) Zeitschrift f. kl. Med., vol. 

xxvi., p. 175. 
Gowers : vol. ii., p 674. 

Kitasato : Zeitsch. f. Hygiene, Bd. vii. ; also Deutsch. Med. W., 1889, No. 31. 
Nicolaier : Inaug. Dissert. Gottingen, 1885. 
Nerlich : (Cephalic Tetanus) Arch. f. Psych., vol. xxxiii. 
Rose, Pitha and Billroth : Handb. d. Chir., 1872. 
Runge : Die Krankheit. d. ersten Lebenstage. Stuttgart, 1893. 
Rosenbach: Arch. f. kl. Chirurg., 1887. 
Schultze, F. : Neurolog. Centralbl., 1882. 
Tarnuffi : Centralblat f. Bacteriologie u. Parasitenkunde, 1892. (Quoted in 

an Editorial, Medical and Surgical Reporter, Nov. 2, 1893.) 
Thamhayn : Schmidt's Jahrbiicher, 1861. 
Tizzoni u. Cattani : Berl. kl. Wochenschr., Dec. 4, 1893, and June, 1S94. 



CHAPTER VIII. 

TETANY. 

Tetany or tetanilla is a disease which occurs with far 
greater frequency in Europe than in this country. Osier 
and Weir Mitchell speak of its exceeding rarity in the United 
States. It occurs with about equal frequency in adults and 
children. , As the name indicates, " the little tetanus" is 
characterized by attacks of tonic spasms of various groups 
of muscles, particularly of those of the upper extremities. 
It is only within recent years that the disease has been prop- 
erly studied, though the first account of it goes back as 
far as the year 1830, when Steinheim described this group 
of symptoms as a special form of articular rheumatism. In 
the following year Dance published an article in which he 
expressed the view that the intermittent character of the 
spasms proved the disease to be of a malarial order. The 
name of the disease we owe to Corvisart, who in 1852 re- 
viewed the entire subject. His observations were preceded, 
however, by those of Trousseau, who had observed the 
disease in nursing women, and supposed a connection be- 
tween tetany and the function of lactation. He therefore 
termed it " contractu re rheumatismal des nourrices," but 
later on he observed the trouble in children and in adults 
with intestinal obstruction, and so abandoned the lactation 
theory. It was Trousseau who first discovered the very 
important fact that these attacks could be excited by com- 
pression of the arteries and nerve-trunks of the affected ex- 
tremity. 

Our knowledge of this disease has been enhanced by the 
observations of Chvostek, Koppe, Baginsky, Von Jaksch, 
Bernhardt, and others. The most comprehensive mono- 
graphs on the subject have been written by Weiss, of Vienna, 
in 1 88 1, and by Frankl Hochwart in the same year. 



TETANY. 



161 



Symptomatology. — The symptomatology of tetany in- 
eludes the symptoms to be noticed during the attack and 
those during the period of latency. The attack is preceded 




Fig. 48. — Position of Hands in the Spasm of Tetany. (After Oppenheim,) 

by vague tingling pains, by formication in the hands, fore- 
arms, and legs; a feeling of stiffness soon follows, and subse- 
quently the spasm of the muscles sets in. This tonic spasm 



1 62 THE NERVOUS DISEASES OF CHILD REX. 

occurs more frequently in the upper extremities, and gives 
rise to such a marked rigidity of the muscles that passive 
movements are for the time being impossible. The position 
of the hand during the spasm will necessarily vary accord- 
ing to the groups of muscles affected. It is a common 
occurrence for the hand to assume the shape of the 
accoucheur's hand. Occasionally also the thumb is very 
firmly pressed upon by the flexed fingers and the nails are 
buried in the skin of the palm. In some rare cases there is 
complete extension of all the fingers. The forearms are gen- 
erally flexed, and the upper arms closely pressed against the 
chest. If the lower extremities are involved the thighs may 
be adducted, the legs extended or flexed, while the toes are 
apt to assume the position of talipes equinus. The spasms 
may also affect the muscles of the abdomen, of the chest, 
and of the back. These tonic contractions of the abdomi- 
nal and thoracic muscles may interfere with the movements 
of the diaphragm, and with the respiration, causing dyspnoea 
and cyanosis. If the muscles of the neck be also involved, 
the return of the venous blood from the brain may be re- 
tarded, as reported by Weiss in one case, in which loss of 
consciousness was the result of such neck spasm. Opistho- 
tonus is not infrequent, but trismus is rare and never occurs 
in the beginning of an attack as in tetanus. In severe cases 
spasms of the ocular muscles have been observed, of the 
oesophagus, of the larynx, and of the muscular apparatus of 
the bladder (a desire to urinate but micturition impossible). 
During the attack the patient complains of severe pain in 
the affected muscles ; there is also a diminution of tactile 
sensibility in the extremities, the muscular sense is often 
deficient, and while standing on the floor patients have a 
feeling as though they were walking on velvet. 

Elevation of temperature as high as 104 F. has been ob- 
served ; but this is exceptional, as Weiss records an average 
of only one such case in twelve. 

Headache, vertigo, tinnitus aurium, and excessive perspi- 
ration are other symptoms which have been noted during 
an attack of tetany. The attacks may last only a few 
minutes, or may cover a period of hours, or even days. Se- 
vere attacks of tetany may bear a striking resemblance to 



TETANY. 163 

genuine tetanus ; but it should be noted that there is no ini- 
tial spasm of the masseters in tetany, and that in this form 
the spasm spreads from the periphery inward, and not cen- 
trifugally, as is the case in tetanus. Moreover, the reflex 
excitability, is not nearly as great in tetany as in tetanus. 
In tetany the patients may be entirely free from attacks for 
hours, and even for days, whereas in tetanus the attack, as 
long as it lasts, is continuous. 

Etiology. — The disease occurs chiefly in very young 
persons. The cases observed by Koppe were all from one 
to two years of age. Ganghofner reported 40 cases ; of 
these 5 were between two and three years of age and the 
remaining 35 between the ages of one month and two years. 
Gowers tabulated 142 cases ; of these 64 occurred from one 
to four years, and 36 from ten to nineteen years of age. On 
going over the literature of the subject a few years ago, taking 
all cases of tetany into consideration, it was evident that 
the majority of cases of tetany were observed between the 
ages of sixteen and thirty-five years. At one time the disease 
was classed among the professional neuroses, but this fallacy 
was corrected by Kussmaul. Any exhausting disease may 
be regarded as a possible etiological factor, but exposure to 
cold and wet is referred to most frequently as a predispos- 
ing cause. Intestinal irritation is another cause, and may 
be associated with eclampsia and laryngospasmus, two con- 
ditions which we know are also frequently excited by in- 
testinal irritation. Riegel instances a case in which attacks 
of tetany were inhibited by the removal of the ova of taenia 
medio-canellata and tricocephalus dispar. Weiss reports 
the occurrence of tetany as a complication of typhoid fever. 
It has also been observed together with small-pox, Bright's 
disease, malaria, chorea, and even after severe mental 
shock. The only just inference from all this is that 
tetany is liable to occur after any exhausting disease in 
those who are predisposed to this form of spasm. Weiss 
deserves special credit for bringing into prominence the re- 
lation between tetany and extirpation of the thyroid gland. 
The disease sometimes appears in epidemic form, if we are 
to credit the account of such occurrences in the schools and 
prisons of France. A similar epidemic occurrence is re- 



164 THE NERVOUS DISEASES OF CHILDREN. 

ported by J. Lewis Smith, Escherich, and others. That it 
is much more frequent in some countries than in others has 
already been alluded to. 

The male sex is affected a little more frequently than the 
female ; Rilliet and Barthez recording 20 out of 28 cases in 
boys. In Gowers's statistics of 142 cases, 76 were males and 
66 females. Although the disease sometimes occurs in 
families there seems to me to be insufficient evidence of 
actual hereditary predisposition to this disease. 

Symptoms of the Latent Period. — In the interval 
between the attacks the patient may appear to be entirely 
well, but if examined carefully he will exhibit a weakness, 
with slight rigidity of the affected muscles. The calf mus- 
cles are commonly the seat of mild contractions. Chvostek 
observed slight contractions of the orbicularis palpe- 
brarum. 

The intervals between the attacks of tetany may vary 
in duration from several hours to a few days, and even a 
few months ; but, of course, we can speak of a latent inter- 
val only in case the disease can be proven still to exist. 
This can be done by eliciting Trousseau's symptoms and 
by proving an increased electrical and mechanical excita- 
bility of the parts affected. 

Trousseau 's Symptom. — The famous French physician 
discovered that in persons afflicted with tetany a charac- 
teristic attack can be elicited by pressure upon the large 
nerve-trunks and the arteries of the extremities usually 
affected during an attack. The attack ceases as soon as 
pressure is removed. Kussmaul and Quincke maintain 
that in some cases pressure on arteries only is necessary r 
while in other cases the slightest pressure on a nerve-trunk 
is sufficient to produce contractions of all the muscles sup- 
plied by this nerve. Trousseau's symptom is absolutely 
pathognomonic of this disease. 

Increased electrical excitability has been found to be 
characteristic of tetany by Erb, Chvostek, Weiss, and oth- 
ers. According to these authors the faradic and galvanic 
responses of the motor nerves are enormously increased dur- 
ing the interval between the attacks of tetany. They were 
not only able to obtain kathodal closure contractions (K. 



TETANY. 165 

C. C.) with very small currents, but were able with moder- 
ate currents to obtain a kathodal closure tetanus, and even 
an anodal opening tetanus, which has not been observed in 
any other condition. Chvostek and Weiss claim that this 
phenomenon is exhibited in the facial and in other periph- 
eral nerves. Erb found the electrical excitability greatest 
at the time when the attacks were most frequent. 

Increased mechanical excitability is a still more striking 
symptom of the conditio*??, a simple touch with a percus- 
sion-hammer upon a nerve-trunk being sufficient to pro- 
duce contractions of the muscles supplied by the nerve. I 
remember distinctly the cases in the Vienna General Hospi- 
tal in which pressure with a lead-pencil upon the focal point 
of the pes anserinus was followed by contractions exactly 
like those which a strong faradic current applied to this 
part Avould have produced. This is by far the most con- 
venient test to make in cases in which the existence of tetany 
is suspected, and it is far better to endeavor to establish 
this fact of increased mechanical excitability than to excite 
an attack by pressure upon a large nerve-trunk or a large 
artery. 

Differential Diagnosis. — There can be but little 
difficulty in differentiating between tetany and other con- 
vulsive disorders. From tetanus it can be distinguished 
sufficiently by the intermittent and centripetal character 
of the attacks, by the absence of trismus at the beginning 
of the attack, and, above all, by the presence, during the in- 
terval, of Trousseau's symptom, and the increased mechan- 
ical and electrical excitability. I have known a case of 
frequently repeated epileptiform convulsions to be mis- 
taken for a case of tetany, but the loss of consciousness, the 
universal tonic and clonic movements, the turning of the 
eyes, the stupor following an attack, and the extreme short- 
ness of the attack, are the symptoms that will help to dif- 
ferentiate the epileptiform attack from one of tetany. 

Morbid Anatomy and Pathology. — Post-mortem ex- 
aminations of cases of tetany have been made bv Langhans 
and Weiss, but up to the present dav there have been few 
positive pathological findings. Langhans claims to have 
found a periarteritis and periphlebitis of the white commis- 



1 66 THE NERVOUS DISEASES OE CHILDREN. 

sure and of the anterior horns of the cervical portion of the 
spinal cord. Weiss failed to find any such changes in his 
cases. He formulated an ingenious theory of the disease 
according to which the attacks of tetany are due to an irri- 
table condition of the gray matter of the medulla and spinal 
cord. This irritable condition is the result of sympathetic 
disturbances causing irregularities in the vascular innerva- 
tion of the blood-vessels of the spinal cord. Gowers is in- 
clined to look for the chief changes in the motor cells of 
the spinal cord, but all this is in the nature of speculative 
pathology. Schlesinger has very recently reviewed the 
various theories concerning the nature of tetany. He con- 
cludes that none of them is entirely satisfactory, while, ac- 
cording to his own ideas, tetany is a disease of the entire 
nervous system, some of the symptoms being due to an af- 
fection of the peripheral nerves ; but the tetanic spasms, 
as well as Trousseau's phenomena, are held to be the result 
of increased excitability of the gray matter of the central 
nervous system, chiefly of the brain, medulla oblongata, and 
spinal cord. Peripheral irritants of various kinds may give 
rise to vasomotor disturbances in the spinal cord, and these 
may be the cause of functional changes. This last theory 
is but a little more satisfactory than the preceding ones, for 
nothing is known as yet of that special peculiarity and 
special irritability of the central nervous system which 
give rise to this disease. Irritability of the nervous system 
is so common that something more is needed to explain 
these very unusual manifestations of the disease. That a 
zymotic factor enters into the etiology of tetany can 
scarcely be doubted, but the exact nature of such infection 
is a matter for future study. 

The occurrence of tetany after extirpation of the thyroid 
gland points the way to a future investigation regarding the 
origin of the disease. If it is the function of the thyroid 
gland to eliminate mucin from the body, it is natural to 
infer that the continuance of this toxic substance in the sys- 
tem is responsible for the tetanic spasm that sets in when 
the gland is removed by surgical interference. 

A special caution is in order not to confound ordinary 
carpopedal spasm, or any of the short clonic and tonic 



TETANY. 167 

spasms that so frequently occur in children, with true 
tetany.* The term should be restricted to those cases that 
present distinct attacks and a free interval, characterized 
by Trousseau's symptom and increased excitability. 

Prognosis. — The prognosis is favorable excepting in 
those few cases in which serious lung trouble may result 
from continued spasm of the respiratory muscles ; but the 
prognosis should be very guarded with respect to the dura- 
tion of the disease. It may vary from a few weeks to many 
months. 

Treatment. — The removal of every possible peripheral 
irritation is the first sine qua non of treatment. If intestinal 
irritation is suspected to be the cause, free purging of the 
bowels and removal of intestinal parasites are called for. A 
change of abode may become necessary, and absolute rest 
will have to be secured at any cost. 

To shorten the attack the physician will probably have to 
resort to the hypodermic use of morphine, and possibly to 
hyoscine. Weiss reports a single case in which the attack 
was inhibited by the application of ice to the back of the neck. 
Go wers, who seems to think the spasms of tetany very closely 
related to ordinary infantile convulsions, advises treatment 
similar to that employed in convulsions. Proceding on 
this basis, inhalations of nitrite of amyl and of chloroform 
may be tried. As soon as the attack is over it would be 
well to administer chloral hydrate in daily doses of about 
one-half drachm, or the bromides in doses of from ten to 
twenty or more grains per day, according to the age of 
the child. Small doses of sulfonal, or of trional, are worthy 
of trial. During the interval careful electrical treatment 
(stabile ascending currents through the peripheral nerve- 
trunks) as well as prolonged lukewarm baths should be 
given. There is little doubt but that the majority of cases 
will get well without any therapeutic measures. The chief 
duty that devolves upon the physician is clearly that of in- 
vigorating the central nervous system by the best known 
methods and remedies, in order to enable it to resist the 
ordinary irritants which produce the disease in those who 
are predisposed to it. If the child exhibits any symptoms 

* Some of the cases reported by Vaughan arc not distinct cases of tetany. 



1 68 THE NERVOUS DISEASES OF CHILDREN. 

of rickets, cod-liver oil and iron will be the best remedies, 
and treatment, to be successful, will have to be directed 
entirely to the improvement of the child's defective bone 
development. The bromides may be administered if the 
condition is not intense enough to call for narcotics and 
opiates, and if, for some reason or other, these latter reme- 
dies should not be employed. Gowers states that a dose of 
digitalis, given at bedtime, has been found to be the most 
useful remedy for nocturnal tetany. The action of the digi- 
talis could be explained only on the theory that the blood- 
supply of the central nervous system is deficient, and that 
there is need of increased activity. 



TETANOID CHOREA. 

Under this title Gowers refers to a case which has been under his care 
that exhibited symptoms intermediate between those of chorea and that of 
tetany. The disease ran a fatal course, but no demonstrable lesions were 
found after death. The patient was a boy, ten years of age, with a history 
of three other relatives having suffered from diseases resembling chorea. In 
the patient the symptoms began seven months before death. They consisted 
of tonic spasm, which was continuous, and varied by paroxysmal attacks of 
similar but more intense spasm. The face was involved on both sides so as to 
cause a constant peculiar smile. The tongue was pressed up against the pal- 
ate, impeding swallowing and preventing speech. The arms were extended, 
pronated, and rotated inward so as to bring the back of the forearms outward, 
while the fingers were flexed at the joints, but at times were extended and 
moved slowly in a way characteristic of athetosis. The lower extremities 
were extended at all the joints, the feet being extended in talipes equino- 
varus, and the toes were flexed. The muscles of the trunk were also in- 
volved in the spasm. At first the left side was the more severely affected, 
but afterward the spasm became equal on the two sides. 

The electrical irritability of the muscles was normal, and there was no 
mechanical excitability of the nerves. There was considerable pyrexia during 
the more severe stage of the disease. The boy became thoroughly emaciated, 
and died from, exhaustion. There is very little of true tetany, and still less 
of chorea in this case as described by Gowers. While I have seen no case 
exactly like this one of Gowers, it is not rare to find irregular spasmodic 
movements of all sorts that may remind one at times of chorea, at times of 
athetosis, and then again of a cataleptic condition during febrile disorders 
in children. 






TETANY. 169 



BIBLIOGRAPHY. 

Abercrombie : On Tetany in Young Children. London, 1890. 

Baginsky: Tetanie bei Sauglingen, Arch. f. Kinderh., 1886, vii., H. 3. 

•Chvostek : Zeitschr. f. kl. Med., vol. xix., H. 5 u. 6. 

Ewald: Allgemeine Med. Zeitung., 1893, lxii., p. 489. 

Erb : Electrotherapie. 2d Ed. 6. 188 

Eulenburg : Tetanie, Eulenburg's Encyclopaedia. 

Frankl, Hochwart : Jahrbuch f. Psych., ix. ; Deutsch Arch. f. kl. Med., 1886, 
xliii. 

Griffith, J. P. C. : Tetany in America, with a Report of Five Cases, Trans- 
actions of the Association of American Physicians, 1894, ix. 

Hoche : Versuche mit Curarin bei Tetanie, Neurolog. Centralbl., April 15, 
1894. 

Hoffman : Deutsch Arch. f. kl. Med., 1888, xliii., H. 1. 

Von Jaksch : Ztschr. f. kl. Med., 1890. 

Pvifgel : Deutsch Arch. f. kl. Med., vol. xii. 

Sachs, B. : Article Tetany, in Wood's Reference Handbook. 

Schlesinger, H. : Versuch Einer Theorie der Tetanie, Neurolog. Centralbl., 
1892, p. 66. 

Schultze : Deutsch Med. Wochenschr., 1882, Nos. 20, 21. 

Vaughan : Some Cases of Tetany, Medical Journal, Dec. 24, 1893. 

Weiss : Volkmann's Sammlung, No. 189, 1881. 






CHAPTER IX. 

HEADACHES. 

It requires no little skill on the part of the physician to 
discover the true cause of headaches. They do not consti- 
tute a form of disease, but are symptomatic of many or- 
ganic and functional conditions. For this reason we must 
give them the very closest study. The diagnosis of the 
kind of headache in the adult is difficult enough, but it is 
doubly so in the case of children. As the headaches that 
come on in early life are frequently continued in later 
years and give rise to much suffering, it is particularly im- 
portant that an effort should be made to nip them in the 
bud. The most distressing form of headache and the one 
most stubborn in its resistance to treatment, migraine, will 
be considered in a separate section. The remaining forms 
may be classified as follows : 

Headaches due to : 

i. Anaemia and malnutrition. 

2. Neurasthenia. 

3. Transitory hyperaemia, as it occurs in heart disease 
or at the period of menstruation. 

4. Gastric disturbances. 

5. Genital irritation. 

6. Ear disease. 

7. Organic disease of the brain or its coverings (includ- 
ing specific disease). 

8. The prodromal stage of acute infectious diseases. 

9. Malaria. 

10. Uraemic poisoning. 

n. Other toxic conditions. 

It was the fashion formerly to make a differential diag- 
nosis of the forms of headaches according either to the 






HEADACHES. 



171 



character of the headache or to the seat of the distribution of 
the headache. Thus frontal headaches were considered to 
be due to gastric disturbances. Occipital headaches were 
considered to be almost pathognomonic of uraemic poison- 
ing ; but he who has an opportunity of seeing a large number 
of cases of headaches in the adult, as well as in children, will 
soon convince himself that these signs are often misleading. 
I have seen frontal headaches with disease of the kidneys, 
and occipital headaches from gastric disturbances and in 
cases of specific disease, of tumor of the brain, etc. The diag- 
nosis of the kind of headache can, as a rule, be made after 
carefully examining into the general health of the patient, 



Constipation ) 
Caries of Incisor ) 



Errors of Refraction 



Gastric Dyspepsia i 
Anaemia J 




Eye 

Decayed Teeth 



( Pharyngitis 
1 Otitis Media 



Fig. 49. — Location of Head-pains. After Dana (slightly altered). Area I. Trigem- 
inus and Facial Nerve Strands. Area II. Upper Four Cervical Nerve Strands. 

and endeavoring to find what the fundamental disturbance 
may be. It will be better for us, therefore, to take the eti- 
ological conditions mentioned above, and to endeavor to 
connect with each condition a few signs that are generally 
associated with a particular form of headache. 

1. Headaches due to anaemia. These headaches occur 
frequently in children between the ages of eight and fifteen 
years ; they are sometimes frontal, generally vertical, and 
are described, as a rule, as a dull, boring headache, and in the 
majority of cases are most pronounced earlv in the morn- 
ing. They are frequently attended by a slight vertigo and 
a feeling of faintness, but none of these symptoms is char- 
acteristic enough to make the diagnosis of anaemic head- 
ache unless the headaches due to eve-strain, to neurasthenia. 



172 THE NERVOUS DISEASES OF CHILDREN. 

or to urasmic conditions can be excluded. The pallor of 
the conjunctivae, which may be obscured by a catarrhal 
condition very frequent in children, and above all the pal- 
lor of the gums and of the roof of the mouth, and a defi- 
ciency of red blood-corpuscles are the signs which help to 
make the diagnosis of anaemic headaches certain beyond 
peradventure. 

The result of treatment will also furnish corroborative 
evidence. The child should be placed at rest, fatigue 
should be avoided in every possible way, and its diet should 
be nutritious to the extreme. It is important not only to 
give nutritious food, but to exclude everything that is of 
no decided value to the child's condition. If we allow an 
excess of fruit or of sweetmeats the child will very natu- 
rally look for these rather than for meat, fish, eggs, oysters, 
which should constitute its main diet, with a slight admixt- 
ure of farinaceous substances. One to two quarts of milk 
per day, three or four eggs, a pound or more of meat will 
be all of far more benefit to the child than any number of 
drugs. They may help a little, but I am certain that it is 
only a very little; and if there is any question of the child's 
power of assimilating these drugs I much prefer to give 
them up rather than to diminish the amount of food that 
the child takes. In the treatment of children's diseases the 
practical necessity of prescribing something often puts 
physicians at a great disadvantage. The era of rational 
therapeutics has not yet fully dawned. 

These anaemic headaches will be relieved in some in- 
stances by the proper use of hydrotherapy. It is a good 
plan to bathe these children every morning immediately 
upon rising. Sponge the head and spine first with warm 
water and then with cold, allowing the water to drip from a 
little height, so as to get the effect of the impact of cool 
water. After this they should be briskly dried and allowed 
at once to dress. In the case of very weak children it 
would be better to have the child take a little nourishment, 
say a glass of hot milk, before putting it through this pro- 
cedure. In addition to this the child should be given 
regular calisthenic exercises, which help to improve the 
general condition and to develop the muscles, and to pre- 






HEADACHES. 1 73 

vent the accumulation of fat, which is not infrequent in 
children of anaemic disposition. 

The meals should be given at regular intervals, and in 
cases in which the appetite is poor the plan of giving small 
quantities at frequent intervals, say every two hours, is to 
be preferred to that of only three meals during the day. 

The most useful drug to be employed in the treatment of 
these headaches is caffeine in doses of one to two grains 
every hour, or at least three times a day. Black coffee will 
often answer the same purpose. From the combination of 
phenacetine and caffeine I have seen very little benefit, and 
it surely has no advantage over the administration of caf- 
feine alone, and is possessed of the disadvantage that phe- 
nacetine acts unfavorably upon the hearts of some children. 
If iron is to be given let it be administered in the most 
easily digested form, either in the form of reduced iron, or 
in one of the many preparations of the peptonates or albu- 
minates of iron that are now in the market. Arsenic can 
be given in small doses of from three to four drops of the 
Fowler's solution several times a day. In cases in which a 
general tonic effect is desired, we can give a palatable com- 
bination of iron and quinine with small doses of strychnine, 
from one-eightieth to one-sixtieth of a grain, three times a 
day. Why phosphorus should be recommended by some I 
cannot understand. I am certain that no one can conscien- 
tiously assert that he has seen any good effect from its use, 
and whether given in pill form or in the form of Thomson's 
solution, the effect in my experience upon cases in which I 
have known it to be tried has been absolutely nil. The 
fewer the drugs employed, and the more attention is paid to 
the hygiene and the diet of the child, the more quickly 
this anaemic condition will disappear. 

2. Headaches due to neurasthenia. These headaches 
occur so frequently in persons who are at the same time 
anaemic, that the treatment of one cannot be considered 
apart from the treatment of the other. In anaemic persons 
headaches very often do not occur until they have been 
subjected to some emotional excitement, or have passed 
through some fatiguing ordeal. These headaches can be 
recognized as due to some other cause than to anaemia 



174 THE NERVOUS DISEASES OE CHILDREN. 

alone by the presence of other symptoms expressive of the 
neurasthenic state, such as slight tremor of the tongue and 
fingers and the exaggeration of the deep reflexes, partic- 
ularly the knee-jerks ; but above all by the very, persistent 
location of these headaches on the crown of the head, and 
by the description of them as a pressure or a feeling of heat 
on the top of the head. This description of the headache 
is as characteristic for children as it is for older patients ; 
and in children emotional conditions, the strain of school 
work, the rivalry between classmates, is quite as apt to bring 
about a neurasthenic state and to cause neurasthenic head- 
aches as are the more serious struggles for existence in 
later life. 

The treatment of neurasthenic headaches may be con- 
ducted on exactly the same lines as was referred to for 
those due to anaemia. The hydrotherapeutic procedures 
and rest are by far the most important, and strychnine and 
quinine in small doses will be of far more benefit than if 
anaemia were the only cause ; but a cure cannot be easily ef- 
fected unless all exciting conditions are removed, and to that 
end the child should be taken from school, and in some 
instances a change of climate and of home surroundings 
may be necessary. If this is not sufficient a short rest in 
bed with forced feeding may be attempted, for the " rest- 
cure" plan is as efficient in children as it is in adults. 

3. Headaches due to transitory hyperaemia. Fluctua- 
tions in the blood-supply of the brain sometimes cause 
severe headaches in children. I have seen many young 
persons who after some unusual excitement or intense emo- 
tion develop severe headaches, associated either with deep 
flushing, or with marked pallor of the face and with cold 
extremities. In others, again, persistent headaches come on 
after an acute exhausting disease, in whom it must be sup- 
posed that if there is any transitory hyperaemic condition 
it is rapidly followed by a lack of blood in the vessels of 
the brain and its coverings. A chronic hyperaemic condi- 
tion is probable in children afflicted with heart disease, who 
are often subject to periods of painful headaches, and a 
similar explanation must be sought for in young girls just 
beginning to menstruate, who have severe headaches for 



HEADACHES. lj$ 

several days preceding and during each menstrual period. 
While such headaches are often strictly periodic they are 
very different from ordinary migraine, and can be distin- 
guished from the latter, too, by the late onset of the trouble 
and by their disappearance as soon as the menstrual func- 
tion has been properly established. 

4. Headaches due to gastric disturbance are a very 
common occurrence in children of all ages. They are 
generally frontal, sometimes frontal and vertical. They 
come on suddenly and persist, as a rule, until the gastric 
condition has been relieved. These headaches are easily 
recognized by the symptoms associated with them, viz., 
fetid breath, coating of the tongue, distress in the epigas- 
tric region, flatulency, and constipation or diarrhoea. The 
diagnosis is easily corroborated by the effects of treatment, 
a single dose of oil or of calomel being sufficient, as a rule, 
to dispel them until the next serious error in diet is com- 
mitted. 

5. Genital irritation is referred to by some writers as a 
cause of severe headaches. Seguin refers to a case of oc- 
cipital headache, the worst he ever saw, which was cured 
by circumcision. I have not seen such a case, but I do 
not wish to doubt their occurrence, and if the physician is 
convinced of the cause of the disturbance the remedy is 
close at hand. In older boys, and even in girls, headaches 
are not infrequently due to self-abuse. The general nervous 
condition of the patient under these circumstances calls 
for treatment even more distinctly than do the headaches 
which are associated with a general restlessness, with pains 
in the back, with irritability, and in severe cases with a 
tendency to mental apathy or even dementia. There is 
every reason to look for this etiological factor in any case 
of persistent headaches in children between the ages of six 
and fifteen years. The treatment of the headaches under 
these circumstances is not an easy matter, and practically 
they can be cured only by the closest watchfulness on the 
part of parent or nurse. 

6. The headaches associated with ear disease are char- 
acterized by intense pain, located either in the mastoid or 
in the temporal region, and are most frequently present in 



1/6 THE NERVOUS DISEASES OF CHILDREN. 

the earlier stages of the disease, before the formation of 
pus. One of the worst cases of this sort that I have ever 
seen was in a little girl, two years of age, who was un- 
manageable and showed by her movements that she was in 
great distress. Every touch on the head was followed 
by a shrill cry, and the region of the ear was so sensitive 
that the slightest touch with the finger seemed to cause in- 
tense agony. No treatment was of any avail until after a 
few days the pus was freely discharged through the outer 
canal, and from that moment every trace of headache 
seemed to have disappeared. The child's behavior is 
exactly like that of an adult with acute ear disease, only 
that in children this special cause of headache is often dis- 
covered only by the merest accident. The practitioner and 
the specialist in ear diseases will meet with these cases 
so often, particularly in connection with the acute infec- 
tious diseases, that they should be on their guard. The 
cure depends entirely upon prompt treatment of the ear 
condition. 

7. Headaches due to organic disease of the brain or its 
coverings should be suspected in every case in which the 
pain is persistent and strictly localized. The pain may 
vary a little according to the position of the head, but it is 
present whether the child be sitting up or lying down, and 
is always elicited by the gentlest percussion of the skull. 
The headaches constitute a most valuable symptom in the 
diagnosis of tumors of the brain or of the meninges, and 
in cases of meningeal inflammation from any and every 
cause. Thus after slight traumatic injuries to the head the 
onset of intense pain, particularly at the seat of external in- 
jury, must lead one to suspect the development of a mor- 
bid condition at this point. If due to tumor, the diagnosis 
can be strengthened by the discovery of other symptoms, 
which are bound to arise sooner or later. These are ver- 
tigo, nausea, and disturbances of vision due to optic neuritis. 
If a traumatic meningitis is the cause of pain in the head, 
slight rigidity of the neck, and possibly an inequality and 
immobility of the pupils, will help to prove the diagnosis. 

Among the organic headaches we might also include 
those due to specific disease ; but these headaches, if I may 






HEADACHES. 1 7/ 

trust my own experience, are relatively rare in cases of 
hereditary syphilis or in syphilis acquired at a very early 
date. They are surely not nearly as constant nor so im- 
portant a symptom as are the specific headaches of later 
years. The diagnosis should be made with great reserve, 
and only if other symptoms are present which point to an 
active syphilitic process. 

8. The headaches which mark the prodromal stage of 
acute infectious diseases often give rise to very serious er- 
rors in diagnosis, and are occasionally suspected to be symp- 
toms of cerebral tumor, or possibly of meningitis. In the 
prodromal stages of typhoid and scarlatina, and of diph- 
theria, these headaches are very common indeed ; but the 
possibility of such a cause for headaches should be enter- 
tained if they are associated with a general malaise and 
with slight rise in temperature. There is no need of dis- 
cussing the treatment of this special form. Unless one 
chooses to apply cold cloths, or an icebag, or possibly to 
give small doses of phenacetine, there is nothing else to do 
but to wait for further developments, and to treat the more 
serious disease of which the headaches constitute the pro- 
dromal stage. 

9. Malarial headaches are not observed nearly so often 
in this climate as in the more southern States, and in 
those countries in which severe types of malaria are prev- 
alent. Malarial headaches are almost invariably neuritic in 
character. The supraorbital and infraorbital points are 
painful, and the entire head may be sensitive to touch. 
There is a distinct periodicity in the development of these 
headaches, or if the headaches are continuous there are at 
least periodic exacerbations. 

In making a diagnosis of malarial headaches I follow the 
practice of examining the spleen and the blood, and not 
contenting myself with the diagnosis unless I can prove 
the existence of enlarged spleen, or the presence of the Plas- 
modium in the blood. If such evidence is obtained, a few 
large doses of quinine — from five to ten grains several times 
a day, according to the age of the child — will prove the best 
cure. 

10. Uraemic headaches are not as frequent in children as 

12 



178 THE NERVOUS DISEASES OF CHILDREN. 

in the adult. These headaches are generally occipital, and 
are associated with slight disturbances of vision, with ver- 
tigo, nausea, and sometimes with epigastric pain. The con- 
dition of the kidneys should be carefully determined, if 
severe headaches arise during or after any of the acute in- 
fectious diseases ; above all, in scarlatina and diphtheria, 
which are known to be followed by renal complications. 

11. Other toxic headaches deserve special mention. 
They are not frequent, but do occur often enough to make 
it necessary to examine for this possible cause. Among 
toxic substances lead is most easily productive of severe 
headaches. The poison is apt to be taken into the system 
not only with milk that is kept in leaden jars, but with 
drinking-water passing through leaden pipes, and I have 
known it to result from chewing-gum, and all sort of vile 
sweetmeats that are wrapped up in attractive papers, the 
children licking these papers in order that they may get 
the full value of their purchase. Not long ago I saw at my 
clinic a young girl, of about fifteen years, suffering from 
headaches, and from a typical lead palsy, who had evidently 
taken the poison into her system in the course of her daily 
work, which consisted of gluing together the paper in 
which chewing-gum was wrapped. She was in the habit 
of putting her tongue to the glue and the paper instead of 
moistening the former with a sponge. 

I have reserved for the last, the consideration of head- 
aches due to eye-strain. Not that I think them the least 
frequent, but because in my opinion undue importance has 
been attached to them. The cases that are due to eye-strain 
are those in which the headaches come on after reading, or 
in studying. They may persist for some time after the 
effort is made, but frequently disappear after the effort is 
relaxed. Serious errors of refraction may be the cause of 
headaches, and of continuous headaches, even though no 
effort be made to use the eyes ; but I have seen headaches 
persist so frequently after the fitting of glasses by the most 
competent oculists that I am firmly convinced that eye- 
strain is the sole cause of headaches in relatively few in- 
stances. 

These headaches are located in the frontal region, be- 



MIGRAINE. 179 

tween and over the eyebrows (Fig. 49) ; in some instances 
with evident eye-strain the headache is referred to the occi- 
put. I am thoroughly in favor of giving every child the 
benefit of the doubt, and of making a careful examination 
into the condition of the eyes ; but I wish to protest against 
the excessive enthusiasm of the day which implies that if 
the slightest error of refraction is discovered in a child the 
error must be the cause of all ills. What is claimed for 
headaches is claimed under similar conditions for epilepsy 
and for chorea. 

The above considerations will convince the student that 
the diagnosis of headaches is no easy matter. To make an 
accurate, or even plausible, diagnosis of this condition is 
one of the most difficult tasks in neurology. It can be done 
properly only by a careful consideration of the general con- 
dition of the patient, of the symptoms associated with the 
headaches, and of the patient's health before and after 
the headache has been developed. 

MIGRAINE. 

Among the neuroses of early youth few are more 
troublesome or more interesting than migraine, or "sick 
headache." While the disease does not, as a rule, attain its 
full development until the age of puberty or later, it begins 
so often in the earlier years of life that it belongs very 
properly to the special subjects of this book. The symp- 
toms of the disease as it occurs in children are so very much 
like the adult form that in describing one we picture the 
other. 

Hemicrania (megrim), or sick headache, is characterized 
by occasional attacks of intense headaches, frequently unilat- 
eral, which are associated with a feeling of nausea, or with 
vomiting. The unilateral headache is by far the more im- 
portant symptom of the two, for in many cases the charac- 
teristic headache is present for months and years without 
nausea ever being associated with it, though I must record 
cases from my own experience in which periodic attacks oi 
nausea have occurred in children without any headaches. 
Such attacks I have interpreted to be the equivalent of or- 



l80 THE NERVOUS DISEASES OE CHILDREN. 

dinary migraine. The correctness of this view has been 
proved by the occasional occurrence of attacks in which 
both nausea and headaches were present, and by the fact 
that the ordinary treatment for migraine and no other 
helped to dispel these peculiar gastric attacks. Barring 
such occasional cases, it is better for us to consider the 
typical attack in which neither headache nor nausea is 
wanting. 

In this disease we find a number of very important symp- 
toms associated with headache. Peculiar visual disturbances 
constitute a prominent feature of the disease ; these have 
been variously described by many sufferers, and amount, 
as a rule, to a temporary and partial loss of sight during the 
attack ; or there may be every possible form of visual dis- 
turbance, from simple balls of fire to distinct figures, which 
appear as regularly in the attacks of migraine as similar 
phenomena do in attacks of epilepsy. In many cases a sim- 
ple dark spot is observed ; in others, flashes of lightning 
that surprise the patient are the first symptoms of a full- 
fledged attack of migraine. A young patient of mine would 
regularly see a bright zigzag line, which she compared to 
a distant staircase ; as soon as she seemed actually to ap- 
proach the stairs intense headaches would set in, and she 
would feel dizzy, but there was never loss of consciousness, 
and nothing resembling an epileptic attack. The patient 
would then pass through a typical attack of migraine, which 
would last for several hours, and during this entire time 
would exhibit very marked photophobia. In some cases 
other special senses are affected. Tinnitus is much more 
frequent, on the whole, than any other form of sensory 
disturbance excepting those of vision noted above. 

Disturbances of sensation in the limbs are a frequent ac- 
companiment of migraine. These sensations generally take 
the form of tingling, of pins and needles, or of burning sen- 
sations. In some instances there is a general numbness, 
very much like the numbness that precedes an attack of 
epilepsy ; the sensations, however, last very much longer 
than the sensory aura would, and can, of course, be distin- 
guished from the latter by the entire absence of any typical 
clonic movements. Motor symptoms are not present as a 



MIGRAINE. 1 51 

rule. As there is a close association, at times, between mi- 
graine and epilepsy it is possible that we may see cases 
every now and then in which the auras resemble very much 
those that precede an attack of migraine, but the clonic 
movements are characteristic of epilepsy. 

Temporary aphasia I have met with as an accompani- 
ment of an attack of migraine. I recall the case of a young 
girl, ten years old, who had inherited migraine from her 
mother, and who greatly alarmed her parents by the sudden 
development of aphasia in connection with an attack of mi- 
graine. The girl when spoken to was able to mumble a few 
words indistinctly, but could not find the word she wished 
to say. She was in intense pain and extremely irritable, 
but, after a good night's rest, the headache had disappeared 
and with it the aphasia. This aphasia is associated with 
right-sided hemiplegia in right-handed persons. The occur- 
rences during these attacks of migraine adhere closely to 
the physiological laws of the cortex, and we may anticipate 
what we have to say upon the pathology of the subject to 
the extent of implying that the sequence of symptoms evi- 
dently proves that the entire motor district of the brain 
must be affected by the temporary defect in its blood- 
supply. 

Vertigo is another symptom that is associated with the 
headaches, at times preceding it, at other times following 
it. The vertigo is not, as a rule, as marked as it is in Me- 
niere's disease or in some organic diseases of the brain, but 
it is quite sufficient to make the patient unsteady on his- 
feet, and to give rise to a great deal of discomfort. 

The aspect of the patient varies considerably during the 
attack. In some there is distinct pallor of the face and a 
feeling of coldness in the extremities. The eyeball may 
seem a trifle retracted, the vessels of the conjunctiva may 
be engorged, and the pupils may be contracted. In other 
cases the face may be extremely flushed, the pupils dilated 
rather than contracted, and the ear distinctly reddened. 
These two distinct conditions are often present in one and 
the same attack, and, in rare instances, the one-half of the 
face may present pallor with its associated symptoms. 
whereas the other half presents a flushed condition with 



1 82 THE NERVOUS DISEASES OF CHILDREN. 

the symptoms that go with it. The latter symptoms are 
evidently of the paretic order and the former of the spas- 
tic, and both are evidences of a change in the sympathetic 
nerves. Sweating of one side of the face has also been ob- 
served, as well as retardation of the pulse during the parox- 
ysms. On examination during an attack, I have found the 
various points of the trigeminal nerve quite as sensitive as 
in the milder cases of trigeminal neuralgia. In some pa- 
tients — and this is particularly true of children — the entire 
face and head is so sensitive that the patient can scarcely 
bear to rest the head on a pillow, and I have known one 
patient who insisted on " walking off " the headache rather 
than to rest the head against anything during the parox- 
ysm. 

Gowers refers to a transitory pyrexia during an attack 
of migraine in children. I have often taken the tempera- 
ture during attacks, impelled to do so by a flushed condition 
of the face, but have never found any elevation above ioo° 
F. If a higher temperature is present I should be disposed 
to look for some other condition, say some gastric disturb- 
ance, as a possible cause both of the migraine and of the 
fever. 

Etiology. — The hereditary predisposition to migraine 
is too manifest to be denied for a single moment. Not only 
do children of mothers who are thus affected inherit the 
disease, but it also occurs in the progeny of persons afflicted 
with other grave forms of functional nervous disturbance. 
Among these epilepsy, hysteria, and hystero-epilepsy are 
the most potent sources. The disease usually sets in in the 
earlier years of life. Some begin between the fifth and tenth 
years, a fair number of the cases between ten and twenty 
years, but the majority set in between twenty and thirty 
years. 

The female sex is evidently more disposed to the disease 
than males. 

The first manifestations of migraine are generally excited 
by emotional disturbances, by overwork, or worriment. In 
not a few instances I have known the rivalry among class- 
mates to have been the final cause of the development of 
migraine ; in others, some severe gastric disturbance has 






MIGRAINE. 183 

been the starting-point of the entire trouble, but it should 
be remembered that, if a predisposition did not exist, the 
derangement of the stomach might have produced a tem- 
porary headache, but would not have started a series of 
attacks of migraine. After the first attack an interval of 
some weeks, or months, may intervene before a second 
seizure takes place ; but some form of periodicity is soon 
established in the majority of the cases, and in many, even 
in young children, the attacks are apt to return at stated 
intervals — every two, every four weeks, or every second or 
third month. It is with migraine very much as it is with 
epilepsy, that the slightest disturbance of the physical or- 
ganism is sufficient to develop an attack. It is, therefore, of 
the greatest importance in migraine, as in epilepsy, that the 
most careful attention should be paid to the general hygi- 
enic and dietetic management of the child. 

Pathology. — We cannot expect to demonstrate actual 
changes in the brain, or in any other part of the central ner- 
vous system in a person afflicted with migraine, for persons 
so afflicted rarely die after a paroxysm, and the attack of mi- 
graine evidently represents a transitory change ; but since 
the anatomical basis is wanting, the opportunity for theo- 
retical speculations regarding the pathology of the disease 
is all the greater, and has been improved by numerous 
writers. There can hardly be a doubt that changes in the 
blood-supply of the brain or its coverings are primarily 
responsible for the symptoms of migraine. There is also 
reason to believe that the sympathetic nervous system is 
largely involved in this disease. According to the varying 
conditions present many neurologists are inclined to sup- 
pose that in some instances we have an angio-spastic condi- 
tion, in others an angio-paralytic state, and if we wish to 
imply our belief in the role played by the sympathetic 
nerves we can speak of " sympathetico-tonic " or " svmpa- 
thetico-paralytic " forms. The behavior of the superficial 
blood-vessels in the two forms of migraine lends reasonable 
coloring to this view of the vasomotor origin of migraine. 

Some, not satisfied with this vascular theory, argue that 
there must be some inherent alteration in the nerve-cells of 
the brain, but no sufficient reason is given why. if such de- 



1 84 THE NERVOUS DISEASES OF CHILDREN. 

rangement exists, there should be such violent periodic ex- 
acerbations of all the symptoms. To say that the symp- 
toms are due to a " nerve storm " is merely substituting a 
vague term for a vague conception ; yet we must acknowl- 
edge that a peculiarity in the structure or in the function 
of the cortical cells may be present in these cases, and that 
changes in the vasomotor apparatus are sufficient to pro- 
duce the phenomena of migraine in a person whose nervous 
system is thus altered, whereas the same vasomotor changes 
would be entirely insufficient to produce any such symptoms 
in persons whose brain -cells are altogether normal. It 
seems to me, therefore, that we must rely upon these two 
causes for an explanation of the disease ; surely one cause 
alone would not be sufficient to explain all the phenomena. 
We are constantly losing sight of the co-operation of forces 
and of causes that produce disease, and are hampered so 
frequently by the supposition that we must make out a 
single cause or none. 

The disturbances of vision, as well as the temporary 
aphasia occurring in some cases, prove conclusively that 
the cortical centres are involved in the disease. The visions 
of migraine can be regarded as a symptom of irritation 
the hemianopsia as a symptom pointing to temporary paral- 
ysis of the functions of the visual centres. Whether a de- 
rangement of the cells or some other change in the consti- 
tuent parts in these centres is responsible for the loss of 
function cannot yet be proved or denied. 

The relation of migraine to epilepsy makes it also more 
probable that some primary peculiarity of the cortical cells 
is responsible for this painful affection. The resemblance 
is so close, and the sequence of the two diseases so strik- 
ing, that the two forms of disease may possibly represent 
a different degree of affection of the cortical structure. 

As for the headaches of migraine, they can be best ex- 
plained, it seems to me, on the supposition of a marked 
distention of the blood-vessels of the coverings of the brain ; 
and the fact that various points in the face are as painful as 
they are in typical trigeminal neuralgia, would lead to the 
inference that the trigeminal nerve, as it passes from the 
brain outward, is affected by this general hyperasmic con- 



MIGRAINE. 185 

dition. The nausea and vomiting are an expression of gen- 
eral cerebral disturbances, such as is seen in cases of gross 
disease anywhere in the brain, particularly in the lower 
centres in the pons and medulla, and such as we sometimes 
find in cases of simple cerebral shock. I cannot see suffi- 
cient reason to connect these symptoms directly with an 
affection of the sympathetic nerve. 

Within the last few years much has been made of ocular 
insufficiencies as a possible cause of migraine. This matter 
has been much overdone, thanks to the labors of Dr. Stevens 
and others. As a neurologist I could pass over the rather 
elaborate discussion that has been held on this subject, 
were it not for the fact that so high an authority as Dr. 
Seguin has given his approval to this special doctrine in 
his lectures on the treatment of neuroses {New York Med- 
ical Journal, 1890). Dr. Seguin states that he has not met 
with a case of migraine in a person with normal eyes, al- 
though he has been told of two or three by oculists of good 
repute. He implies, furthermore, that if the matter has 
been overlooked in the majority of cases of migraine, it is 
because the ocular examination has not been a thorough 
one. He argues, furthermore, that the ocular origin of 
migraine is made probable by the remarkable fact that in 
many persons of both sexes the attacks diminish and then 
cease between the ages of forty and fifty years, at the 
time the power of accommodation becomes exhausted, and 
a large part of the unconscious strain which has been going 
on from early youth is removed. Seguin implies still further 
that migraine is hereditary, chiefly because ocular defects 
are hereditary. The fallacy of these arguments seems to 
me to be evident enough from the experience many of us 
have had, that the eyes are normal according to the exami- 
nation of competent oculists in many children who have 
migraine, and that the attacks continue long after the ocu- 
lar difficulty has been corrected by glasses. We are willing 
to concede that these ocular difficulties have an important 
bearing upon headaches in general, and may influence the 
occurrence of attacks in those who have inherited migraine ; 
but far stronger evidence will have to be advanced before 
it will be safe to adopt Seguih's conclusions, and sonic other 



1 86 THE NERVOUS DISEASES OF CHILDREN. 

sufficient reason will have to be given for the action of 
mydriatics, such as belladonna, atropine, hyoscyamia, and 
cannabis indica, than the effect these have upon the accom- 
modative effort. In my own experience with the drugs 
mentioned, cannabis indica is the only one which has any 
decided effect upon the course of migraine or upon single 
attacks. 

Diagnosis. — The diagnosis of migraine is easily made. 
The early onset of the disease, the periodic attacks, and the 
character of the headaches will, as a rule, leave little room 
for doubt. It is only in those cases in which a distinct sen- 
sory aura exists that a confusion with epilepsy might arise, 
but the preservation of consciousness and the entire absence 
of clonic movements, and the duration of the single attack, 
will help to distinguish migraine from epilepsy. It may 
be a little more difficult at times to distinguish between 
migraine and headaches due to some other cause, but this 
difficulty will cease as soon as the periodicity in the occur- 
rence of the headaches has been noted, and every other 
form of headache has been ruled out by the method of ex- 
clusion. I have found some little difficulty also, particu- 
larly in young children, to distinguish between migraine 
and ordinary trigeminal neuralgia, for owing to the sensi- 
tiveness of younger patients it is not so easy to determine 
whether there are distinct painful points, or whether the 
whole face is painful, merely as a result of the fear of ex- 
amination ; but the long and free interval between attacks, 
together with the periodic occurrence of the headaches in 
migraine, will serve to distinguish this form from typical 
trigeminal neuralgia.* 

Prognosis. — The prognosis of migraine is entirely fa- 
vorable as regards life, but not so promising with reference 
to the cure of the disease. It is a matter of common ex- 
perience that a person who has inherited migraine will be 
subject to the disease for a long number of years, until it 
begins to disappear with age. It is a satisfaction, however, 
to be able to assure the patient that under proper treatment 
the attacks may be either inhibited or their severity may be 
diminished. The only grave feature about the disease is 

* Trigeminal neuralgia is a rare affection in children. 






MIGRAINE. 187 

the possible development of migraine into epilepsy, but 
even this is an infrequent occurrence. 

Treatment. — In the treatment of migraine two distinct 
objects must be kept in view. First — the cure, if possible, 
of the disease ; secondly — the amelioration of the attacks. 

The disease often defies the skill of the most experienced 
practitioner. As in epilepsy, no effort should be spared 
to accustom the patient to regular hygienic and dietetic 
habits. It is important that the child thus afflicted should 
sleep in a well-ventilated room ; that it should have regular 
and moderate physical exercise, a point of the greatest im- 
portance in those who have inherited gouty or lithaemic 
tendencies. The child should be relieved also, as far as 
possible, of all mental and physical strain. While the dis- 
ease is at its worst the patient should be kept from school, 
and all close application to study should be avoided. If it 
can be shown that the effort of reading or of studying helps 
to bring on an attack, even study at home and reading may 
have to be prohibited. As a matter of fact, however, a 
little mental occupation is often to be preferred to idleness, 
which permits the child to wait for the onset of an attack 
and often induces distinct hypochondriacal tendencies. My 
own experience has been very largely in favor of mod- 
erate employment of mind, which helps to prove to the 
child that its lot is not a worse one than that of other chil- 
dren, and helps also to avoid the idea of invalidism which is 
so frequent in children, even in those who do not for one 
reason or another care to simulate disease. 

If the child is anaemic, or if it exhibits scrofulous ten- 
dencies, iron in any of its various forms, arsenic and quinine 
in tonic doses, as well as cod-liver oil, are indicated. Phos- 
phorus is of so little use that we can disregard it alto- 
gether. 

A single attack can be relieved best by putting the 
child in bed during the period of the attack, and in a dark- 
ened room. On the whole mere rest in bed is as important 
a factor as any in the treatment of the attack. During this 
time the child should be placed on a mild diet ; an excess oi 
liquids as well as of nitrogeneous food should be avoided. 
Small amounts of meat, fish, and eggs, and a moderate 



1 88 THE NERVOUS DISEASES OF CHILDREN. 

amount of milk, will answer the purpose better than if the 
patient is placed altogether upon nitrogeneous or upon an 
excess of farinaceous food. 

It is important to regulate the bowels thoroughly, and 
if the kidneys are not very active to increase the quantity 
of urine passed. To this end some mild alkaline water, 
such as Vichy or Seltzer, can be safely recommended, 
either alone or in conjunction with milk. 

Innumerable drugs have from time to time been warmly 
recommended. Some years ago antipyrine in doses of five 
to ten grains, according to the age of the patient, was quite 
in vogue ; but I da not consider it promising enough to 
urge its use in the face of the well-known risks attending 
its exhibition. I am certain, too, that phenacetine in five to 
ten-grain doses has little or no effect. I have seen some good 
results from the combined action of the phenacetine and 
caffeine in one- to three-grain doses, but I am certain that 
the combination is not in any way superior to the use of 
caffeine alone. Caffeine is beyond a doubt one of the most 
serviceable drugs to be employed. I am in the habit of 
giving it to children under fifteen years of age in one-grain 
pills or powders, which are to be repeated every twenty min- 
utes until distinct relief is felt ; and after the first relief has 
been procured the drug should be continued in the same 
dose at longer intervals, first every two, then every three 
hours, and later on, for a period of about a week after the 
onset of the attack, it can be given safely in small doses 
several times a day, according to the condition of the 
child. Caffeine can be combined with iron, with quinine, or 
with arsenious acid. I have also had excellent results from 
a combination of caffeine and cannabis indica. The various 
preparations of the latter are so unreliable that it is as well 
to use Herring's extract (gr. T V - -J-). A caution is neces- 
sary, however, as regards the use of cannabis indica, for 
some patients are peculiarly intolerant toward this drug, 
and I have seen distinct symptoms of poisoning from small 
doses repeatedly employed. While the drug is not in 
reality a dangerous one, the symptoms due to the admin- 
istration of large doses are so disagreeable and are so apt 
to alarm the patient and the family that great care should 






MIGRAINE. 189 

be exercised in the use of the drug ; but if the fact of 
tolerance on the part of the patient has once been estab- 
lished no trouble is apt to ensue during subsequent trials. 
Seguin thinks that the drug exerts a sedative, even paralyz- 
ing, influence upon the third cranial nerve and its attached 
muscles, including the ciliary, but the drug has as good an 
effect in cases in which the ocular apparatus has been de- 
termined to be entirely normal. 

The nitrite of amyl has been suggested on physiological 
grounds as a proper remedy, particularly in those cases in 
which there is marked pallor of the face. We might also 
expect it to act favorably, as it does in epilepsy, in those 
cases of migraine in which there is a distinct sensory aura. 
I quite agree with Dr. Seguin in the statement that the 
drug is of little use in actual practice, and that it rarely 
affords the expected relief. The same can be said of acon- 
itia and of hypodermic injections of hyoscyamine. The bro- 
mides and chloral help at times to allay the nervous excite- 
ment and to induce sleep, as do also sulfonal and trionaland 
chloralamid in five- to fifteen-grain doses ; but I cannot re- 
gard them as in any sense specific remedies in migraine. 
Nitro-glycerine (one drop of the one per cent, alcoholic 
solution) has occasionally helped to diminish the severity of 
an attack. In this form it is not a dangerous drug, and can 
be safely administered under the supervision of a physician, 
of a nurse, or of an intelligent mother. The use of tea 
and coffee in moderate quantities is strongly to be recom- 
mended ; I have known children suffering from migraine 
keep simple black coffee in the stomach with beneficial 
effect who would vomit almost everything else that was 
given. I feel that I owe much to Dr. Seguin's suggestion 
that black coffee, without sugar and without milk, is easily 
digested, and that the general prejudice regarding this 
beverage is due to the milk and sugar that have been in- 
variably added, and that have given rise to fermentative 
processes in the gastro-intestinal tract. 

As for the correction of the ocular defects, I do not 
wish to oppose sensible treatment by competent oculists, 
but I do oppose treatment based upon extreme theories. I 
would have the eye condition corrected on the principle 



190 THE NERVOUS DISEASES OF CHILDREN. 

that in migraine, as in epilepsy, every physical defect 
should be remedied ; but I must protest against the as- 
sumption that such slight errors in refraction and in ac- 
commodation may be the cause of this neurosis. The 
more liberal our therapeutic view, the more successful the 
treatment Avill be. 

BIBLIOGRAPHY. 

(A bibliography of headaches it is quite impossible to give. Many text- 
books on diseases of the nervous system treat the subject very fully ; in 
addition to these, the reader is referred to Seguin's article in Keating's Cyclo- 
paedia, and to one by Sinkler in Pepper's System.) 

MIGRAINE. 

Charcot: Lecons du Mardi, i., 1887. 

Collins, J. : New York Medical Record, April 14, 1892. 

Eulenburg : Article " Migraine," Ziemssen's Cyclopaedia ; Wiener Med. 
Presse, 1887. 

Fere: Ophthalmic Migraine, Rev. de Med., 1883. 

Haig: British Medical Journal, 1888. 

Hammond, G. M. : Journal of Nervous and Mental Diseases, 1892. 

Liveing : On Megrim, Sick Headaches, and some allied disorders, etc. Lon- 
don, 1873. 

Mitchell, S. W. : American Journal of the Medical Sciences, vol. 94, 1887. 

Moebius: Die Migrane, Vienna, 1894. (Contains recent literature; an ex- 
cellent monograph appeared after this chapter was written.) 

Oppenheimer, H. S. : Boston Medical and Surgical Journal, vol. 119, 1888. 

Ranney: "Eye-strain," etc., Medical Record, 1889. 

Sinkler : Medical News, July, 1890. 

Thomas, L. : La Migraine, Paris, 1887. 



CHAPTER X. 

THE DISORDERS OF SLEEP. 

The healthy new-born child sleeps during the entire day 
and night, except when it is being nursed or dressed. Dur- 
ing the first month the infant is awake only three or four 
hours out of the twenty-four, falling soundly asleep imme- 
diately after nursing. By the end of the sixth month fifteen 
hours' sleep per day is a fair average, which is gradually de- 
creased until at the age of one year the healthy child sleeps 
about twelve out of the twenty-four hours. From one year 
to four years an average of ten hours' sleep should be main- 
tained, and up to the age of ten or twelve years the health of 
the child demands that it shall sleep quietly for at least 
nine hours in the day. If there is a marked departure 
from these averages, the child is either in pain or some of 
its functions are not entirely normal. In the acute fevers 
or in any febrile condition children are alternately drowsy 
and wakeful. Disturbances of digestion are also a frequent 
cause of restless sleep or of prolonged wakefulness. 

In addition to these special conditions, others cause in- 
somnia in children as in adults. Poorly ventilated and over- 
heated rooms often cause restlessness, and for this reason it 
is important that a window should be kept open in every 
bedroom during the entire night, and that the child should 
be covered as lightly as possible, according to varying at- 
mospheric conditions. If a child is sleepless in the absence 
of unfavorable conditions, we must seek some other cause 
for the insomnia. In many cases the mother or nurse has 
not been careful to engender the proper habits of sleep. 
Infants that are much pampered, that are taken up as soon 
as they utter the first cry, or that are wilfully disturbed by 
parents or nurse, soon become poor sleepers. From the 
first week of life the child should be laid down quietly and 



192 THE NERVOUS DISEASES OF CHILDREN. 

not rocked to sleep, and should not be disturbed except for 
good reasons. If the room is darkened a quiet sleep will 
come on very naturally. As the child grows older it is 
well not to excite it just before the hour at which it is ex- 
pected to fall asleep. With children up to the age of eight 
years the evening meal should be a light one, and their 
brains should not be disturbed by exciting stories. This 
should be observed more particularly in the case of chil- 
dren who exhibit nervous tendencies, or of those who have 
passed through exhausting diseases. 

The normal child does not fall asleep unless its hunger 
has been appeased. Insufficient nourishment is, therefore, 
to be suspected in cases of persistent wakefulness in young 
infants. With the change of nurse, or after weaning, a 
restless sleeper will often develop normal habits of sleep. 
If insomnia continues without apparent cause, we must sus- 
pect some graver trouble. In the earlier stages of tubercu- 
lar meningitis, in tumor, and in other painful diseases of the 
brain, sleeplessness is often one of the earliest symptoms. 

If the cause of insomnia cannot be easily made out, it 
is well to fall back upon faulty methods of training. Not 
very long since a child of five years was brought to me by 
its mother, who stated that it would not fall asleep if it 
awoke after midnight. The mother claimed to be ignorant 
of any mistake that she had made in the training of the 
child, but on closer questioning I heard that she had been 
in the habit, whenever her husband was away, of taking the 
child out of its bed and putting it in her own bed. After 
she had done this a number of times the child woke up 
regularly at midnight and would cry until the mother took 
it up. The mother, a very nervous woman, excited the 
child by her caresses and her despair over its not sleeping, 
and thus made matters worse and worse. The child had 
been sleeping very poorly for several weeks before I was 
consulted. The cure was a very simple one. The child 
was placed under the care of a sensible trained nurse, who 
would not yield to its solicitations, and after a few nights 
of restless sleep it began to develop proper habits and soon 
slept an average of ten hours. 

The prognosis is invariably favorable unless the condi- 



THE DISORDERS OF SLEEP. 1 93 

tion is due to some grave disorder. It is most important 
to insist upon absolute regularity in putting the child to 
bed at a definite hour and keeping it there unless it is neces- 
sary to take it up. If this should prove insufficient a warm 
bath at bedtime will be conducive to sleep, and small doses 
of bromide or chloral will be useful to engender the habit. 
The newer hypnotics, such as sulphonal or trional, in five- 
to ten-grain doses, may be substituted for the bromide or 
chloral in children who are anaemic or poorly nourished. 
All hypnotics should be withdrawn as soon as possible, 
for there is great danger of accustoming the child to these 
drugs. In some cases the mere regulation of the meals, and 
the avoidance of all indigestible articles of diet, will be suffi- 
cient to restore sleep. In children who are impressionable 
it is of the greatest importance to avoid telling them stories 
or even engaging them in play in the latter part of the day. 

Pavor Nocturnus, or night fear, is a condition which 
is often troublesome both to the parents and the physician. 
Children affected with this disturbance wake up a few 
hours after they have entered into a sound sleep, are pos- 
sessed as a rule by great fear, fail to make out their sur- 
roundings, and act as though in a temporary dazed con- 
dition. They do not understand the soothing words of 
parent or physician, and often continue in a state of excite- 
ment until they return to full consciousness or are over- 
come by sleep. 

There seems to be no good reason to give any other in- 
terpretation of this condition than that the child has had a 
horrible dream, and that the substance of the dream is con- 
tinued in the half-waking state, or that the child is possessed 
by fear, which is the natural result of the dream and can- 
not shake off the fear until it fully realizes, if old enough, 
that its experiences have been unreal. 

This condition may occur in any child, but it is decidedly 
more frequent in children who have shown previous nervous 
symptoms, or in children of highly neurotic parents. It is 
not infrequent in those who are pressed by school duties. 
and who go to bed with a fear that their lessons have not 
been properly done for the next daw This knowledge is 
sufficient to prevent sound sleep, and during the hours oi 
13 



194 THE NERVOUS DISEASES OF CHILDREN. 

restlessness vivid dreams are very apt to occur. It was 
formerly supposed that late and indigestible meals were 
the chief cause of these noctural disturbances. Inasmuch 
as any indigestion may give rise to restless sleep, this factor 
may have to be taken into account ; but over-work, over- 
excitement, the reading of horrible or fascinating tales just 
before bedtime, constitute a much more important etiologi- 
cal factor. 

These noctural attacks often occur a single time, but 
with most children are frequently repeated during the ear- 
lier years of life, say between the ages of three and eight 
years. With the growth of the child and the improvement 
of its nervous system the attacks cease. 

The prognosis of this condition is entirely favorable ; 
though I can recall one case of a girl, aged six years, the child 
of an hysterical mother, in whom the fright connected with 
one of these nocturnal attacks was the cause, or at least the 
first beginning, of a severe chorea, which lasted for many 
months. In another child, about nine years of age, who had 
had attacks of epilepsy ever since early childhood, and who 
was particularly subject to nocturnal attacks of epilepsy ; 
the epileptic attacks ceased upon bromide treatment, but 
every now and then the child would have a very marked 
spell of pavor nocturnus, which would disturb her almost 
as much as an epileptic seizure would. 

Conditions similar to those of pavor nocturnus occur at 
times during the day. A little patient of mine, aged five 
years, would, in a very unaccountable manner, while walk- 
ing with her mother on the street, suddenly hide her face in 
the folds of the mother's dress, and bury her head for some 
time, in order (as she explained later on), not to see the hor- 
rible black things coming toward her. This child showed 
no other symptom of a nervous disposition, but was for a 
time in great dread of the recurrence of these experiences. 
That they were the result of visual hallucinations during 
the waking hours, I can hardly doubt. They disappeared 
entirely under sedative treatment, which was given carefully 
for some months. 

In regard to treatment, the most important point is to ex- 
ercise every possible precaution in order that unnecessary 






THE DISORDERS OF SLEEP. 1 95 

nervous excitement, late in the day, may be avoided. I 
prohibit all school-work after five in the evening ; do not 
allow the child to read or be read to after this hour, and in- 
sist on a very light meal in the evening. After this meal 
the child is to remain awake for at least an hour, and may 
indulge in some simple game, or is allowed to frolic about 
in a quiet manner. Small doses of the bromides, from five 
to ten grains, according to the age of the child, about an 
hour before bedtime will be the best remedy. If neces- 
sary, small doses of chloral may be given with the bromides. 
If the attacks return very frequently I have found it more 
satisfactory to give five- or ten-grain doses of chloralamid, 
or of trional, every night, or every second or third night, 
until all excitement has subsided. 

Enuresis Nocturna is another very common form of 
disturbed sleep. Children who have been well trained, 
and who have learned to observe all the habits of cleanli- 
ness during the day, are frequently disturbed by involuntary 
micturition during the night. This is most apt to occur 
during the earlier hours of sleep, when sleep is most pro- 
found. The soundness of sleep alone may be a sufficient 
cause for this phenomenon, inasmuch as children do not 
perceive in sleep those symptoms of vesical irritation which 
prompt them to evacuate the bladder during waking hours, 
and during light sleep. In others, and possibly in the ma- 
jority of cases, it is due to indifference on the part of the 
child, or to an aversion to allow its sleep to be interrupted. 
In some few cases enuresis is the result of frightful dreams, 
the sudden impulse to evacuate the bladder accompanying 
such a dream as it would any emotional condition during 
wakefulness. If due to this latter cause the enuresis is not 
frequently repeated. Enuresis has been observed to be a 
symptom of nocturnal epilepsy. If so, it occurs at rare in- 
tervals ; the child is entirely unconscious of it, and often 
shows some other symptom, such as drowsiness and head- 
ache on the following morning. 

The condition is a very stubborn one, and all possible 
methods of treatment have been suggested. I am con- 
vinced that the cure of the condition is dependent chiefly 
upon careful training. The necessity of evacuating- the 



10 THE NERVOUS DISEASES OE CHILDREN. 

bladder should be impressed upon children after the age 
of two years. The child should be made to void urine im- 
mediately before going to bed, and should be aroused one 
and a half to two hours after it has fallen asleep, and should 
be induced to pass water again. By giving relatively few 
liquids after four or five o'clock in the afternoon, I have 
found that the tendency to enuresis is often checked. 

If these simple measures are not sufficient, a few drops 
of the tincture of belladonna, given at bedtime, will be use- 
ful, and if the condition is due to frightful dreams, or to 
cerebral excitement continued during sleep, small doses of 
bromides may be given. Many medical men have seriously 
suggested that corporal punishment applied to the nates is 
the only efficient remedy, and some have gone so far as to 
suggest that the cutaneous hyperaemia caused by such pun- 
ishment explains the relief afforded. This explanation is 
not satisfactory ; but whatever the mode of action may be, 
it is very certain that such punishment need not be inflicted 
in the 'majority of cases, and that the desired end can be 
attained by other methods of training. 

Somnambulism is by no means rare in children, and occurs most fre- 
quently in those who are of a nervous temperament, or who have passed 
through some severe excitement. I have not known them to perform 
any of the marvellous tricks which are generally accredited to somnambulists, 
such as climbing out of windows and on the roof, and the like ; but they are 
apt enough to walk through the house, from one room to another, and to go 
through a number of purposive actions without being at all conscious of what 
they are doing. Nightmare is evidently closely related to this condition of 
somnambulism, and is generally associated with horrible dreams, from 
which the child awakens badly frightened, but is quieted much more rap- 
idly than in the condition which is described as pavor nocturnus. Night- 
mare and somnambulism occur, as a rule, at rare intervals and can generally 
be traced to some emotional excitement, or to some acute indigestion. The 
treatment of these conditions is exactly the same as that of night-terror. 

BIBLIOGRAPHY. 

Lyman : Insomnia and other Disorders of Sleep. Chicago, 1885. 

Putnam, C. P. : Article in Keating's Cyclopaedia (literature to date, 1890). 

Ringer: Medical Times and Gazette, 1867. 

Silberman : Jahrbuch fr. Kinderheilkunde, 1883. 

Soltman : in Gerhardt's Handbook, p. 325. 

Also the text-books of Henoch, Baginsky, and West. 



CHAPTER XL 

VASOMOTOR AND TROPHO-NEUROSES. 

In this chapter a brief description will be given of a 
number of rare and peculiar diseases, which must for the 
present be classified under the rather vague title of vaso- 
motor and tropho-neuroses. Grouping them together un- 
der one head is simply a matter of convenience ; they are as 
dissimilar as diseases can well be. Raynaud's disease and 
facial hemiatrophy have nothing in common ; myxcedema 
and Graves's disease very little ; moreover, myxcedema must 
now be removed from the category of " neuroses," if by 
the latter term we mean a disease of the nervous system 
with unknown organic changes. The nervous system is in- 
volved secondarily, and the marked changes underlying the 
symptoms are well known. In former days several of 
these vasomotor disorders were considered under the head- 
ing of diseases of the sympathetic nervous system ; but 
the sympathetic has nothing to with myxcedema, and very 
little with Graves's disease, or facial hemiatrophy. But if 
they differ in the marked changes underlying them, they 
have at least this in common, that, in all of them, changes in 
the skin and the subcutaneous tissues constitute the most 
striking symptom. 

EXOPHTHALMIC GOITRE. 

This disease, which is generally termed Graves's disease 
by the English writers, and Basedow's disease by the Ger- 
mans, occurs chiefly in adult life, yet a sufficient number of 
cases begin during childhood to make it incumbent upon 
us to give a brief description of the disorder. Moreover, 
two conditions resembling Basedow's disease, viz.. tachy- 



198 THE NERVOUS DISEASES OF CHILDREN. 

cardia and enlargement of the thyroid in girls at the age 
of puberty occur often enough to make it necessary to dif- 
ferentiate between these various conditions. Von Ehrlich 
and Kronthal have called special attention to the occur- 
rence of exophthalmic goitre in children. 

The chief causes of Graves's disease in adult life are un- 
questionably great emotional excitement, some constitu- 
tional or exhausting disease, and occasionally some serious 
cardiac disturbance, but no cause is as frequent as fright or 
emotional excitement. In children these same factors may 
be at work, but heredity comes much more distinctly into 
play, particularly in children of persons suffering from 
epilepsy, chorea, or of those suffering from chronic al- 
coholism. 

The symptoms of this disease are very numerous. We 
may distinguish between the cardinal and accessory symp- 
toms. The cardinal symptoms are : 

1. Excessive cardiac action. This is by far the most 
constant symptom of all, and is often present in the earlier 
stages of the disease when the other two cardinal symptoms 
— swelling of the thyroid and the protrusion of the eyes — are 
not yet in evidence. The pulse-rate may vary between 90 
and 120, or it may even reach the enormous rate of 200. 
Under great excitement the pulse-rate is increased. Under 
mental and physical rest the pulse-rate may not exceed 90 
or 100. In spite of the acceleration of the pulse-rate the 
other signs do not point to any organic disturbance of the 
heart. The percussion figure is normal, with only this ex- 
ception, that in the later stages of the disease the left ventri- 
cle, and rarely the right ventricle, may become hypertro- 
phied and dilated. The heart-sounds are clear but unusu- 
ally loud. All the arteries pulsate very distinctly, so that 
the pulse of the abdominal aorta may possibly be observed 
through the abdominal walls, and the pulse in the carotids 
may be so strong as to cause distinct pulse-like vibrations 
of the entire head. The pulse may even be noticeable in 
the retinal arteries, and a further disturbance of the entire 
vascular system may be noted by a dilatation of the capil- 
laries in the skin, by the presence of distinct tdches cere- 
brates, and a dilatation of all the larger superficial veins. If 



VASOMOTOR AND TROPHO-NEUROSES. 1 99 

venous stasis is marked, cedema may occur in various parts 
of the body. It is also owing to the increased tension that 
hemorrhages are frequent from the nose, and even in the 
stomach and bowels. Cerebral hemorrhages have also been 
described in Graves's disease. A particularly characteristic 
symptom, which is found in exophthalmic goitre, and not 
in any form of enlargement of the thyroid, is the whirring 
felt if the finger is placed upon the goitre. 

2. The second cardinal symptom is the enlargement of 
the thyroid gland. It is present in the vast majority of 
the cases, though a number of authors have taken pains to 
describe cases of this vasomotor disease in which the 
thyroid was not enlarged ; but the enlargement, as a rule, 
comes on some time after the tachycardia. The gland, after 
it has once become enlarged, increases rapidly, and may give 
rise to a great deal of inconvenience. The enlarged gland, 
or struma, is extremely vascular, and the tissue of the gland 
is not only hyperaemic but very soon enters into a state of 
hyperplasia with a tendency to a fibrinous degeneration. 
In keeping with these changes the goitre may in the begin- 
ning be soft and yielding, later on it will become hard to the 
touch. The enlargement of the thyroid is, as a rule, bilateral, 
but generally asymmetrical. The right half is more fre- 
quently enlarged than the left half. 

3. The third cardinal symptom is exophthalmus, or pro- 
trusion of the eyes. This is the least constant of the three 
symptoms. If present it is generally bilateral, but in some 
instances one eye only may be affected, or the protrusion of 
one may be very much greater than that of the other eve. 
The causes of this protrusion have been in dispute, but it is 
more than probable that the interference with the venous 
current, as well as the arterial congestion, are the prime 
causes of this protrusion. The great variability of this 
symptom during life, its disappearance almost immediately 
after death, and the pulsation of the vessels in the fundus, 
lend support to this view. It can hardly be doubted, how- 
ever, that in some cases the development of fat and of cellu- 
lar tissue in the retro-bulbar space may makejthe protrusion 
still more marked. 

Vision is not affected in spite of the exophthalmus, and 



200 THE NERVOUS DISEASES OF CHILDREN. 

the only changes observed were those recorded by Kast 
and Willbrand, who have noted a limitation of the field of 
vision in this disease without any evidence of hysteria. The 
pupils may be unequal, but react promptly. Ulcerations of 
the cornea, occurring in consequence of insufficient protec- 
tion of the bulb, have been noted ; as well as some interfer- 
ence with the lachrymal secretion. 

Among the accessory symptoms those relating to the 
movements of the eyes are the most important. Graefe's 
symptom refers to defective movements of the upper eye- 
lid on vision downward. The upper lid does not follow 
promptly the downward movement of the bulb, and the con- 
junctiva remains visible between the pupil and the margin 
of the lid. This symptom evidently bears some relation to 
the phenomenon described by Stellwag consisting of a dila- 
tation of the palpebral fissure. Graefe's symptom, as far as 
can be ascertained, is caused neither by the protrusion of 
the eyes nor by a spasm of the levator or a paresis of the 
orbicularis, but is explained most readily as the result of 
Stellwag's phenomenon. Moebius has described another 
symptom implying a deficiency in the power of accommo- 
dation. There is defective convergence of the axes of the 
eyes. These three symptoms are not absolutely pathogno- 
monic of Basedow's disease, as each one has been described 
in connection with other neuroses; but if they are associated 
with either one of the cardinal symptoms they help to cor- 
roborate the diagnosis of Basedow's disease. 

The next most frequent, and perhaps most important, 
symptom is a tendency to profuse diarrhoea. This is en- 
tirely independent of any gastro-intestinal disease, often 
resists treatment, and adds greatly to the patient's discom- 
fort and to the general depreciation of his health. Gastric 
crises have been described in some cases, and frequent 
vomiting is not an unusual symptom. Most patients exhibit 
considerable dyspnoea, resembling true asthma, and Louise 
Bryson has referred to defective expansion of the chest on 
inspiration. Glycosuria and albuminuria have been re- 
ported ; the former, according to the investigations of Chvo- 
stek, is of the alimentary order. In some few instances, 
however, diabetes mellitus has occurred as a complication 



VASOMOTOR AND TROPHO-NEUROSES. 201 

of Basedow's disease. This association is not a strange one, 
since both diseases are often developed after severe emo- 
tional excitement. Of the disturbances in menstruation we 
need take no account, as we are, after all, concerned with 
the disease as it occurs in children. 

Trophic and vasomotor disturbances of the skin are fre- 
quent. Pigmentation, leukoderma, loss of hair, premature 
grayness of hair, and even scleroderma, as well as herpes, 
have been observed. A very remarkable symptom of 
Graves's disease is a tendency to profuse sweating. This 
may be universal or partial, sometimes strictly unilateral. 
This tendency must be held to account for the remarkable 
diminution in electrical resistance which has been observed 
in the skin of patients suffering from this disease. This fact 
was first insisted upon by Vigouroux, and later by Eulen- 
burg. Kahler and others have shown that this reduction in 
resistance to the galvanic current is due to the fact that the 
relative minimum of resistance is reached much more easily 
in patients suffering from Graves's disease than in healthy 
individuals. Kahler has also shown that this diminished 
resistance is not due to an hyperemia, as was supposed by 
some, but it is due to excessive moisture of the skin. A 
faint tremor is frequently observed, and by some is con- 
sidered to be one of the cardinal symptoms, but it is not as 
constant as the three cardinal symptoms we have mentioned. 
The tremor is rhythmical and consists of eight or nine vibra- 
tions per second. Choreiform tremor occasionally occurs, 
and true chorea and epilepsy are complications met with 
in a certain number of cases. These complications are in 
all probability purely accidental, and cannot readily be ex- 
plained on the supposition that the vasomotor changes in 
Basedow's disease are the cause of the chorea and epi- 
lepsy. 

In addition to the preceding symptoms mental changes 
deserve some notice, and among these we may note irrita- 
bility of temper, sudden changes of temperament, and the 
occasional occurrence of mania or melancholy. Some oi 
these psychic changes are developed in the earlier stages of 
the disease, others are later, and possibly accidental compli- 
cations. If we add anaemia, albuminuria, and a general 



202 THE NERVOUS DISEASES OF CHILDREN. 

cachexia, we have completed the list of the symptoms typi- 
cal of Graves's disease. 

The morbid anatomy of Graves's disease has been the 
subject of much discussion. The cervical sympathetic has 
been given a very important part. A number of authors 
have reported actual changes in the cervical ganglia as the 
result of connective-tissue proliferation, and others have re- 
ported atrophy and degeneration of the nerve-elements ; but 
since Hale White has proven that similar changes occur in 
persons who have not exhibited the symptoms of Graves's 
disease, and that in persons who have died from this disease 
such changes have not been found, the force of all these 
findings has been much diminished. The same may be said 
of hemorrhages into the fourth ventricle, or atrophy of the 
corpus restiforme, to which Mendel and Leube have attached 
some importance. The heart has naturally exhibited hyper- 
trophy or dilatation, insufficiency of the mitral valve, and 
symptoms of mild endocarditis, but these changes are 
clearly secondary and do not at least hold any causal rela- 
tion to the disease. 

The theories regarding the pathology of the disease 
have been still more conflicting. Basedow supposed the 
disease due to chlorotic changes in the blood ; but this ex- 
planation is evidently insufficient, as the disease frequently 
affects persons in robust health who are far from anaemic. 
The close resemblance between the symptoms following 
upon section of the cervical sympathetic and those of 
Graves's disease has led many writers to attribute exophthal- 
mic goitre to disease of the sympathetic ! Section of the 
sympathetic produces irritation, and the symptoms follow- 
ing the experiment upon animals are clearly the result of 
irritation ; but we cannot suppose any condition of irritation 
to last for years, and must therefore seek some other expla- 
nation. Friedreich was inclined to the theory that dilatation 
of the vessels is the result of paralysis of the sympathetic; 
that dilatation of the coronary arteries caused tachycardia, 
and that this increased flow of blood to the cardiac muscle 
produced an increased activity of the heart, and that all the 
other symptoms of Graves's disease, the exophthalmus and 
the thyroid swelling, were the result of the arterial hyper- 



VASOMOTOR AND TROPHO-NEUROSES. 203 

semia. Granting that the cardinal symptoms can be ex- 
plained in this way, the many accessory symptoms cannot 
be accounted for on this theory. Other authors have in- 
sisted on the bulbar origin of Graves's disease, and have sup- 
posed that a lesion involving the nuclei of the vagus was 
sufficient to account for all the symptoms, and some post- 
mortem findings have given support to this view. 

All these theories have proved more or less unsatisfac- 
tory, and while some cases may actually be due to one or 
other of the causes mentioned, a thoroughly satisfactory 
theory is still to be established. 

Within recent years a number of authors have insisted 
on the probability of the toxic origin of the disease, and 
have supposed that by the increased action of the thyroid 
gland a toxine is produced which is primarily responsible 
for all the symptoms, but it is questionable whether the 
diseased thyroid produces such a toxine, or whether it is 
not more probable that the toxine is the result of deficient 
function of the gland. 

The course of the disease is, as a rule, extremely chronic. 
The onset may be very sudden, and even violent, but after a 
while the symptoms subside in severity and remain con- 
stant for a long period of years; but the prognosis is not 
necessarily as grave as it is generally supposed to be. I 
have myself seen a number of cases of very marked im- 
provement and of total disappearance of all the symptoms. 
If death ensues it is, as a rule, due to some complicating 
disease, or to extreme exhaustion from diarrhoea, or from 
cardiac weakness, or possibly from acute mania. 

Treatment. — Absolute rest and careful feeding are the 
most important measures. The recoveries which I have 
seen have been in patients who have been placed on the 
rest-cure. The entire freedom from excitement, and regular 
feeding have tended to lessen all the nervous symptoms and 
have influenced the diarrhoeas which are often the most ex- 
asperating and exhausting symptom. The tachycardia is 
remarkably lessened and the dyspnoea is naturally benefited 
by rest in bed. The diet should be simple. It is well to 
avoid stimulants and excitants as well as carbonated waters. 
The excessive dilatation of the stomach, resulting from the 



204 THE NERVOUS DISEASES OF CHILDREN. 

last-named, acts unfavorably upon the heart. Hydrothera- 
peutic procedures, consisting of cold baths followed by 
massage, of douches and drip baths, have been recom- 
mended by many. Mineral baths may have some effect 
upon the constitutional symptoms and thus favorably in- 
fluence the disease. Electricity has been warmly recom- 
mended by many, with special reference to the part played 
by the cervical sympathetic and vagus. To give this 
method a fair trial it is best to place the anode by turns 
over the goitre, the heart, and over the abdomen. The cur- 
rent should be mild and continuous. Recently Rockwell 
has recommended the use of the faradic current. 

As far as medicinal treatment is Concerned the drugs in- 
fluencing the heart's action have been most in favor ; above 
all, digitalis and strophanthus. While these drugs act 
favorably in some cases they unquestionably exert an un- 
favorable influence in others. I have found that exces- 
sive palpitation of the heart could be controlled very much 
better by cold applications to the cardiac region, and by 
the use of the galvanic current, with the anode over the 
heart. I am willing, however, to concede that the effect of 
the latter may be due to suggestion. If there is great 
emotional excitement bromides in small doses may be given 
with advantage. Nitrite of amyl and nitro-glycerine have 
been recommended, but they are to be used with great cau- 
tion, especially in children, and the result is a doubtful one 
at best. Ergot has been administered in some instances, 
and the subcutaneous injections of ergotin into the goitre 
have been practised, but the results have not been favorable 
enough to warrant its continuance. 

Thyroidectomy, or excision of a part of the enlarged 
gland, has come into vogue, and has been reported by 
many German, English, and American writers. Improve- 
ment following the operation has been observed, and in my 
opinion is sufficiently marked to warrant further trial of 
this procedure ; but I am not aware that the operation has, 
as yet, been attempted in children. Sufficient time has not 
yet elapsed to decide whether the relief obtained by the 
operation is merely temporary, or whether a permanent 
cure has been effected. If the enlarged gland presses upon 



VASOMOTOR AND TROPHO-NEUROSES. 205 

the trachea and menaces life, the operation is surely justi- 
fied. 

Thyroid Enlargements at the Age of Puberty. — In connec- 
tion with the consideration of Graves's disease, attention should be directed to 
the occurrence of thyroid enlargement in girls between the ages of twelve 
and fifteen years, a condition for which I have been consulted a number of 
times. At this period the enlargement is often developed quite suddenly, and 
is so marked as to arouse suspicion of incipient exophthalmic goitre. As 
such girls are often anasmic, and have a high pulse-rate the suspicion of 
serious disease becomes still stronger ; but such enlargement of the thyroid 
gland does not continue for any great length of time, and none of the other 
important symptoms of Graves's disease are developed. By attention to the 
general health of the girl, and by direct galvanization of the thyroid gland, 
the symptoms soon subside. 

Tachycardia should also be mentioned, as it may occasionally give rise 
to the suspicion of Graves's disease. An excessively high pulse-rate is fre- 
quent in children, but true tachycardia in children has, to my knowledge, 
rarely been reported. I have seen several interesting cases of this kind, and 
one of them was of unusual interest. It was the case of a boy, of twelve 
years of age, who had been under my treatment for infantile cerebral hemi- 
plegia, and had sufficiently recovered from this condition to be able to go 
about freely, to attend school, and to play with boys of his age. While 
playing on a cold winter's day he was struck in the neck by an icy snow- 
ball. Directly he felt faint, but did not lose consciousness. As soon as he 
was put to bed the tachycardia became evident, and his family physician was 
sent for. From the first day until about three weeks after the accident the 
pulse was at no time less than 200 per minute, and often exceeded this rate, 
so that the number could not well be counted. None of the drugs ex- 
hibited — such as bromides, strophanthus, digitalis, aconitia — had the slightest 
effect upon the heart ; but after a period of about three weeks the symptoms 
subsided quite suddenly, and the boy has been entirely well for several 
years. The injury to the cervical region, and possibly to the cervical sym- 
pathetic, causing tachycardia, is of interest, if we recall the part that the cer- 
vical sympathetic is supposed by many to play in the causation of Graves's 
disease. The treatment of such cases should consist of rest, and the appli- 
cation of an ice-bag to the heart and to the cervical spine ; bromides, heart 
tonics, and aconitia may be tried according to the indications of the case. 

myxcedema. 

Myxcedema is a form of trophic disease to which attention should be 
directed. The disease is of unusual interest, but we need not go into a full 
discussion of the subject, as the one form of it which occurs in children — 
myxedematous idiocy — will be referred to in the Chapter on Idiocy. Myx- 
cedema was first fully described by Gull, in 1873; four years later similar 
cases were reported by Ord, and since that time innumerable authors in 



206 



THE NERVOUS DISEASES OF CHILDREN. 



England, America, France, and Germany have studied this tropho-neurosis. 
Special interest has been attached to this disease, since Kocher described a 
condition following upon removal of the thyroid, resembling myxcedema, to 
which he gave the name, cachexia strumipriva. The Commission that re- 
ported to the Clinical Society of London, in 1888, agreed that myxcedema, 
cachexia strumipriva, sporadic cretinism, represented one and the same mor- 
bid entity. The condition following upon removal of the thyroid gland, 

the results of physiological ex- 
periments (implantation of the 
glands into the abdomen of 
animals — Schiff, Horsley, and 
others), and above all, the brill- 
iant achievements of thyroid 
feeding, have proved conclu- 
sively that deficient action of 
the thyroid gland is the most 
important factor in the etiology 
of myxcedema. 

Symptoms. — Of the symp- 
toms of myxcedematous idiocy 
(the congenital form) we need 
here merely state that the skin 
is glossy or hard ; the lips and 
tongue are thick and large ; the 
hair is stiff and dry ; the child 
is dwarfish in stature ; the men- 
tal condition is that of complete 
idiocy or of imbecility. There 
is always absence of the thyroid 
gland. 

Myxcedema resembling that 
of the adult may come on at 
the age of puberty, or even ear- 
lier. It is characterized by the 
following symptoms : A gen- 
eral increase in the bulk of the 
body ; the skin is firm and in- 
elastic, does not pit upon press- 
ure, and is dry and rough ; the 
folds of the skin disappear, and there is a general obliteration of all the lines 
of the skin, particularly in the face, giving, as a rule, an older and more stu- 
pid expression to the face ; the nostrils and lips are very much enlarged. 
There is distinct apathy and slowness of speech, as well as of action, in some 
instances ; the mental changes include delusions which lead to dementia. 
Albuminuria and glycosuria have been observed ; but these are accidental 
complications, no doubt. 

The disease cannot be mistaken for any other, except possibly chronic 




Fig. 50. — Case of Myxcedema with Idiocy. Pa- 
tient Twelve Years Old : Dwarfish in Stature. 



VASOMOTOR AND TROPHO-NEUROSES. 20 J 

nephritis ; but the lack of pitting, the examination of the urine, and the pe- 
culiar expression of the face will help to remove all doubts. 

The disease is slowly progressive, and may last for ten, fifteen, or more 
years. The prognosis was hopeless until the recent discovery of thyroid 
feeding, and success of this warrants us in rejecting for the present every other 
form of treatment. 

Treatment is to consist of the administration of the pulverized thyroid 
gland of the sheep, which is to be had in all countries. An English prepara- 
tion is, up to the present time, by far the most satisfactory. 

Care should be taken to begin with small doses ; in children one grain 
twice a day should be the maximum dose at the beginning ; this may be in- 
creased to three or live grains twice daily until the amount is established 
which the patient needs to keep in a normal condition. 

A word of caution is in order not to continue the remedy if muscular pain 
and cardiac attacks occur. In two cases I have been compelled to discon- 
tinue the use of the gland on account of extreme and rapid emaciation. 
Tonic measures should be employed promptly if the general health of the child 
has been impaired. 

ANGIO-NEUROTIC CEDEMA. 

Angio- Neurotic (Edema is a trophic disorder, first described by 
Quincke, in 1882, although conditions closely resembling, or identical with it, 
have been known ever since 1827. It has passed under the designations of 
"acute circumscribed oedema," "periodic swelling," "Australian blight," 
"non-inflammatory cedema," etc. 

The disease is characterized by the appearance of circumscribed swell- 
ings in various parts of the body, more especially in the face, throat, and the 
extremities. These swellings appear without any direct cause, and are often 
associated with gastro-intestinal disturbance, which is thought to be due to 
a condition of the mucous membrane of the stomach and bowels similar to 
that of the skin or larynx when these parts are diseased. The disease comes 
on in attacks, which are precipitated most easily by exposure to cold, and by 
slight traumatic injuries. The attacks are most apt to occur between the 
hours of 1 and 5 A.M. In 71 cases the parts first affected were : The face in 
29 cases ; the extremities in 22 cases ; the larynx in 5 cases ; the genitals 
— penis, scrotum— in 3 cases ; the trunk in 6 cases ; the stomach in 3 cases; 
the gums, neck, ear, each in 1 case. 

The cedema may be fully developed within one-half to two hours. The 
color of the skin is either whitish, or a dull roseate hue with a whitish shad- 
ing near the centre of the cedematous patch. There is no pitting on pressure. 
and none of the signs of an inflammatory swelling. The swelling, if in the 
face, may be marked enough to cause disfigurement. The sudden jumping 
of the swelling from one part of the body to another is quite characteristic. 
Subjectively there is a sensation of scalding or burning, or an itching on the 
part affected ; and after the cedema subsides a " heavy wooden " feeling is 
complained of. The disease is not a serious one unless it affects the mucous 



208 THE NERVOUS DISEASES OF CHILDREN. 

membranes of the larynx and pharynx ; if the (Edematous swelling in the larynx 
increases rapidly, death may take place from asphyxia, as in two cases re- 
ported by Osier. One case has been reported that proved fatal from oedema 
of the glottis (Krieger). 

The gastro-intestinal tract, if affected, may exhibit severe disturbance, 
such as pain in the epigastrium, vomiting, colic, retraction of the abdomen, 
and constipation or diarrhoea. 

(Edema of the lungs has been reported as a symptom of this disease ; but 
proof of this is wanting. 

Collins has analyzed 75 cases. 

According to this author the average age at the onset of the disease is 
twenty-seven ; childhood is by no means exempt. Dinkelacker has reported 
the case of a child that had its first attack when three months old ; similar 
cases have been reported by Widonitz and Goltz. The disease occurs nearly 
twice as often in males as in females. It may come on after any exhausting 
condition ; but heredity, as well illustrated in a family described by Osier, is 
the most important factor. In this family five successive generations have 
been affected. Severe emotional excitement and hysterical conditions are 
the forerunners of the disease. The ingestion of certain foods was followed 
by this special form of oedema in cases reported by Osier, showing an etio- 
logical resemblance at least between angio-neurotic oedema and urticaria. 

Matas and others have described cases of this disease coming on after 
or during malarial fever. The diagnosis is made readily enough ; it may be 
confounded with the blue oedema of hysteria, as described by Sydenham, or the 
white cedematous swellings of hysteria described by Charcot ; but the pres- 
ence or absence of the stigmata of hysteria will help to disprove or corrobo- 
rate the suspicion of angio-neurotic oedema. 

There can be little doubt that angio-neurotic oedema is a vasomotor 
neurosis ; it is analogous to the non-inflammatory swellings which Weir Mitch- 
ell described after injury to the peripheral nerves, and to similar swellings, 
which appear after stretching a nerve. I have seen cedematous swellings in 
the face, after section of the trigeminal nerve, very like the circumscribed 
oedema under discussion. The serous exudation in all probability results 
from a retardation of the blood-current, and this in turn must be attributed 
to a local paresis of the vaso-constrictors, or a reflex stimulation of the vaso- 
dilators. 

Treatment of this disease as it occurs in children is more difficult than in 
the adult, in whom the removal of toxic causes, such as alcohol and tobacco, 
is all that is needed. In children the hereditary element is most marked, 
and all we can hope to do is to inhibit the attack and to strengthen the 
general nervous system. To check the attack we may compress the affected 
part by an elastic bandage, or apply dry heat. Small doses of morphia or 
codeia may be given. In the case of swelling in the pharynx or larynx, sur- 
gical interference may be necessary. To improve the general health of the 
child it will be best to administer strychnia in small doses (grain one-hun- 
dredth to grain one-sixtieth), and to give blood-tonics such as iron and ar- 
senic. 



VASOMOTOR AND TROPHO-NEUROSES. 



209 



RAYNAUD S DISEASE. 



Raynaud's Disease, or symmetrical gangrene, is a very rare tropho- 
neurosis, which occurs, however, quite as frequently in children as in adults. 
Indeed, says Morgan, " if there be any period when it is especially prone to 
occur, it is in childhood." Of 93 cases which he analyzed 24 were in children 
under ten years of age, and 5 of Raynaud's original cases were children be- 
tween three and nine years old. Mendel observed a case in a child fifteen 
months old. 

The symptoms of the disease are practically the same as those first enu- 
merated by Raynaud in 1862. A localized ischaemiaor asphyxia in symmetri- 
cally situated parts is the most constant symptom ; this asphyxia may lead to 
gangrene, but does not invariably do so ; nor is the disease invariably sym- 
metrical, and for that reason the designation of symmetrical gangrene is not 
entirely appropriate. The order of development of the symptoms may vary 
somewhat. Before the local syncope is fully developed, there is often a 
general numbness and some pain in the part to be affected. The affected 
area becomes pale and waxy in appearance ; if pricked, little or no blood flows 
from it. This condition may be recovered from, and several such attacks 
may occur, each one ending in recovery ; finally, however, the condition per- 
sists, and local asphyxia is developed ; in some instances the condition is 
reached at once and is persistent. The affected areas become deep red, then 
blue, and finally black in color ; the parts are swollen, and the local temper- 
ature is lowered. Extravasation of blood into the surrounding tissues may 
occur. Recovery at this stage is still possible ; but if the morbid process is 
continued for some time the parts grow thinner, the fingers become atten- 
uated, and finally gangrene results. The gangrene comes on either some 
months after the first symptoms of local asphyxia, or it is developed at the 
earliest period of the disease. The tips of the fingers and toes may be de- 
stroyed by this process, or an entire hand or foot or some other considerable 
part may be destroyed by gangrene. The parts most frequently affected, 
are the fingers, toes, ears, the buttocks, the calves, and the nose. The gan- 
grenous area is generally separated from healthy tissue by a sharp line of 
demarcation ; the dead part may either become mummified, or it may be 
cast off after suppuration. Moist gangrene also occurs at times, with the for- 
mation of large bullae and pus centres. If a gangrenous area heals, it behaves 
like a torpid ulcer (Elliot). 

Intermittent haemoglobinuria and glycosuria have been observed. The 
pathology of the disease is still obscure. Raynaud and his successors were 
of the opinion that the local syncope is produced by a contraction or spasm 
of the blood-vessels (both arteries and veins). The disease of the blood- 
vessels is by several authors attributed to syphilis or Bright's disease. The 
symptoms of Raynaud's disease have also been observed in association 
with tabes, syringomyelia, myelitis, and neuritis. 

The prognosis is generally good ; if death occurs, it is due to sonic in- 
tercurrent disease. Recoveries are frequent, even if local gangrene has 



2IO THE NERVOUS DISEASES OF CHILDREN. 

existed ; but if large portions become gangrenous, the general health of the 
patient may be seriously impaired. In such cases tuberculosis may be de- 
veloped and hasten death. 

Treatment should be directed toward the improvement of the patient's 
general condition, and freedom from all emotional excitement should be in- 
sisted upon. The affected parts should be wrapped in cotton, and placed in 
a position most favorable for the circulation. Dry heat or warm baths may 
be employed to sustain the temperature of diseased areas. Nitro-glycerine 
and nitrite of amyl have been recommended, but they have not been exhib- 
ited successfully. Electric (galvanic) baths, and the application of the gal- 
vanic current through the cervical spine and the affected area have also 
been favored by some ; but very little good has resulted from such treatment. 
The greatest benefit will be derived from surgical measures after gangrene 
has set in. 

FACIAL HEMIATROPHY. 

Facial Hemiatrophy is a rare form of disease. Often it begins in 
childhood, and has important relations to other diseases of the nervous sys- 
tem. It has been described by some with exophthalmic goitre and with mi- 
graine, but its relations appear to me to be very much closer to scleroderma, 
which occasionally precedes it, and I have recently seen a case of universal 
scleroderma in which there was double hemiatrophy of the face. 

The disease is characterized by a distinct diminution in the size and bulk 
of the subcutaneous tissues, and by the alteration in the bony structure as 
well. In one case, on the left side the distance from the middle of the chin to 
the angle of the jaw was 1 1 ctm. ; on the right side, 12^ ctm. ; from the upper 
margin of the naso-labial fold to the middle of the ear was 10^ ctm., while 
on the right side this distance measured 1 1 ^ ctm. The first indications of 
atrophy are found in the skin. A single spot, generally in the cheek, be- 
comes thin and white from the disappearance of pigment. The loss of fatty 
tissue underneath causes a depression also at this point. The atrophy pro- 
ceeds from this and gradually involves the entire half of the face. The eye 
is retracted, there is distinct wasting of one-half of the nose, and even the 
ear of one side may be smaller than that of the other side. In some few in- 
stances a slight glossiness of the skin has been observed, but in the majority 
of cases there is unusual dryness, the condition resembling scleroderma. 
The hair also is apt to be stiff and hard. In some instances the teeth in 
the affected half are small, and undergo decay. There is no disturbance 
of sensation, and no interference with any of the special senses in the ordi- 
nary run of cases. The temperature sometimes varies a little. In a case 
which I reported the temperature was one degree higher in the normal ear 
than in the ear of the affected side. The muscular movements, in spite of 
the increasing atrophy, as a rule, remain normal. Several other authors, 
and myself, have reported cases in which there were distinct tonic and 
clonic contractions of the muscles supplied by the fifth nerve of the affected 
side. Atrophy of one-half of the tongue also occurs in some cases, but it is 
a rare complication. 



VASOMOTOR AND TROPHO-NEUROSES. 211 

The causes of the disease are unknown, except that several cases have 
been developed after traumatic injuries to the face. Thus in one case re- 
ported by Skryme, and quoted by Gowers, in a child three and a half years 
of age, the disease was developed six months after an accident in which "the 
face was jammed and the neck twisted." The disease has also been devel- 
oped after acute infectious diseases, and after other causes which give rise to 
neuritis. There can be no doubt, however, that in many cases the disposi- 
tion to the disease is congenital. 

Pathology. — There was much dispute regarding the origin of this dis- 
ease, some claiming that the cervical sympathetic was responsible, others 
making it a disease of the trigeminal nerve. There is no doubt that a con- 
dition very similar to hemiatrophy will follow upon lesions of the cervical 
sympathetic ; but an autopsy performed by Mendel on a case which had 
been observed by many other men, proved beyond a doubt that in that case, 
at least, the disease was due to a proliferating interstitial neuritis of the left 
fifth nerve, and that this neuritis was most marked in the second branch ; in 
that case the facial nerve was entirely normal, but the left musculo-spiral had 
undergone the same changes as the left trigeminal. My own case, in which 
there were marked clonic and tonic contractions of the masseters, would also 
tend to show that the trigeminus was the chief seat of the trouble, and that 
in some instances the motor, as well as the trophic fibres in the trigeminus 
can be involved in the same morbid process. The occurrence of a neuritis 
of other nerves, as well as the association of facial hemiatrophy with sclero- 
derma, would seem to prove that the morbid process may in some cases ex- 
tend beyond the fifth nerve. 

The course of the disease is, as a rule, steadily progressive, though after 
the lapse of time there may be a complete standstill of all the symptoms. 
The disease does not call for any active treatment, excepting in cases like my 
own, in which the spasmodic contraction of the masseters was extremely 
painful and had to be relieved by opiates. The disfigurement of the face is 
the most serious feature of the disease. In the case of one patient I suc- 
ceeded in rounding out the cheek again by inserting a light rubber plate, 
which was attached by a dentist to the patient's upper teeth.* 



BIBLIOGRAPHY. 

EXOPHTHALMIC GOITRE. 

Booth, J. A. : Journal of Nervous and Mental Diseases, 1894. (Thyroidec- 
tomy.) 
Charcot : Gaz. des Hopitaux, 1893, Nos. 13 and 14. 

V. Ehrlich : Dissertation. Berlin, 1890. (Collection of cases in children.) 
Eulenburg-Guttman : Pathologie des Halssympathicus. Berlin, 1873, p, 32. 
Eulenburg : Berliner klinische Wochenschrift, 1889. Nos. 1 and 3. 

* In view of the excellent results obtained by thyroid feeding in a case of hemi- 
atrophy with scleroderma, this treatment deserves a fair trial. 



212 THE NERVOUS DISEASES OF CHILDREN. 

Eulenburg: Centralblatt f. Nervenheilkunde, 1887, and Centralblatt f. klin- 

ische Medicin, 1890. 
Jacobi: New York Med. Record, 1879, p. 9. 
Kronthal: Berliner kl. Wochensch., 1893, No. 27. (Cases in mother and 

child.) 
Moebius : Zeitschrift fur Nervenheilkunde, vol. i. , p. 460 
Roberts: American Journal of the Medical Sciences, 1876, p. 374. 
Sickinger : Dissertation. Strassburg, 1893. (Operative treatment.) 

MYXCEDEMA. 

Gull: Transactions of Clinical Soc, London, 1874. 

Hammond : Neurological Contributions, I., 1881. 

Hamilton: New York Med. Record, 1882, p. 645. 

Hun and Prudden : Am. Journal of Med. Sc, 1888. 

Horsley: British Med. Journal, 1885. 

Kocher : Arch. f. klin. Chirurgie, vol. xxix. 

Marie: Revue de Med., 1886, p. 207. 

Meltzer: New Yorker Med. Monatsschrift, April, 1894. 

Ord : Transactions of the Medico-Chirurgical Society, London, 1878. 

Reverdin : Revue Medicale de la Suisse romande, Oct., 1882; June, 1883. 

Schiff : Arch. f. Experimentelle Pathologie, vol. xviii., 1884. 

ANGIONEUROTIC CEDEMA. 

Charcot: (Euvres completes. Paris, 1885. 

Collins: American Journal of the Med. Sciences, December, 1892. (Full 

literature.) 
Dinkelacker : Dissertation. Kiel, 1882. 

Elliot : Journal of Cutaneous and Genito-urinary Diseases, 1888. 
Elliot: New York Med. Record, 1891, p. 559. 
Goltz: Deutsche Med. Wochenschr., 1880, No. 17. 
Leyden : Zeitschrift fur klinische Medizin, vol. iv., p. 605. 
Lovett: Boston Med. and Surgical Journal, 1890. 
Matas: New Orleans Med. Journal, Oct., 1887. 
Osier : American Journal of the Med. Sciences, April, 1888. 
Quincke: Monatsschrift f. prakt. Dermatologie, 1882, 1. 
Smith : Med. News, 1889, nv -> P- 3 2 °- 
Starr: New York Med. Journal, Sept., 17, 1892. 
Weiss : Wiener Med. Wochenschr., 1882, No. 14. 
Widonitz : Jahrbuch f. Kinderheilkunde, 1889, N - F-» v °l- xx i x - 

RAYNAUD'S DISEASE. 

Abercrombie: Archives of Pediatrics, Oct., 1886. (In a child aged three 

years alternating with haemoglobinuria.) 
Bramann : Semaine Medicale, May, 1, 1889. (Three brothers, affected at the 

ages of seven, nine, and thirteen years.) 



VASOMOTOR AND TROPHONEUROSES. 21 3 

Elliot : Morrow's System, vol. iii. 
Mills: American Journal of the Med. Sc, Oct., 1878. 
Mendel: Semaine Medicale, 1893. 
Morgan : The Lancet, July, 1 889. 

Pitres et Vaillard : Archives de Physiologie, 1885, vol. v., p. 103. 
Raynaud : Translation by Barlow, New Sydenham Publications. The origi- 
nal These de Paris appeared in 1862. 
Shaw : New York Med. Journal, Dec. 18, 1886. 
Vulpian : Gazette des Hopitaux, 1884, No. 9. 
Weiss: Zeitschrift f. Heilkunde, 1882, vol. iii., p. 233. 

FACIAL HEMIATROPHY. 

Bannister : Journal of Nervous and Mental Diseases, vol. iii., 1876. 
Eulenburg : Ziemssen's Cyclopaedia, vol. xiv., 1877 ; also Zeitschr. f. kl. Med., 

1882. 
Jessup and Brown: St. Bartholomew's Hospital Rep., 1882. 
Lowenfeld : Muenchener Med. Wochenschr., 1888, Nos. 23 and 24. 
Mendel: Neurologisches Centralblatt, 1888. No. 14. 
Mills, C. K. : Pepper's System of Medicine. 
Muraton : Neuroglisches Centralblatt, 1891. 

Newmark : American Journal of the Med. Sc, 1892, vol. civ., p. 294. 
Rosenthal: Berl. kl. Wochenschr., 1889, No. 34. 
Sachs, B. : New York Med. Record, March 15, 1890. 



PART II. 

ORGANIC DISEASES OF THE NERVOUS 

SYSTEM. 



CHAPTER XII. 

DISEASES OF THE PERIPHERAL NERVES. 

SOME PERIPHERAL PALSIES. 

Trumatism, toxic infections, exposure to wet and cold, 
rheumatism, the causes which lead to disease of the pe- 
ripheral nerves in the adult are not nearly as frequent in 
children. When the same causes are operative, however, 
the symptoms do not vary much from those observed un- 
der similar conditions in more advanced years. It will not 
be necessary, therefore, to give the various forms of pe- 
ripheral nerve lesions, of peripheral palsies and neuralgias, 
in full detail. A description will be given of those forms 
which are commonly met with in the earlier years of life. 

A nerve-trunk is made up of a connective-tissue sheath or epineurium, 
which encloses the nerve-fibres proper. These are held together by a finer 
connective tissue, or endoneurium. If properly stained and examined micro- 
scopically, it is seen that each nerve-fibre consists of an axis-cylinder, with 
fine longitudinal striations and surrounded by a myelin sheath. If the nerve 
be teased or lacerated the myelin runs out and collects in drops, behaving in 
every way as a semi-liquid fatty substance would. Like fat it is stained a 
deep black by osmic acid. The myelin sheath is surrounded by a thin con- 
nective-tissue membrane, the sheath of Schwann. The nuclei of this sheath 
are visible at various points. The entire nerve-fibre is divided into segments 
by the nodes of Ranvier. At these nodes the myelin sheath is interrupted, 
and the axis-cylinder is covered merely by the membrane of Schwann. In 
addition to this the nerve-fibre presents the incisures of Lanterman. Blood- 
vessels and lymph spaces complete the nerve bundle. 

The morbid changes in peripheral nerves are either of an inflammatory 
or of a degenerative order ; and the two cannot always be distinguished 
from one another. Inflammation may be restricted to the perineurium (peri- 
neuritis), to the endoneurium (interstitial neuritis), or it may affect the nerve- 
tissue itself (parenchymatous neuritis). In the last-named form the changes 
are very much the same as in degeneration of a nerve-fibre after traumatic in- 
jury, or after any cause which separates the nerve-fibre from its nutrient cell. 



218 



THE NERVOUS DISEASES OF CHILDREN. 



In the vicinity of the actual lesion or of the cut ends (in physiological ex- 
periment and after traumatic injuries) degeneration of the nerve is estab- 
lished. Toward the periphery, the whole of the nerve undergoes degenera- 




o Ml 












c rm 






o In 






u m 






>- Wft 






a; to 






£ ¥ 




*-> \v 






o \ 






h 






<*« 






o 


■£ 




c 






o 


1 rt 






/ OJ 




cd J 


^ 




S / 


m 










£ | 


S 




£ 


IS 


^v 


u 


be 


_<D 


3 

1 


.5 

'3 


a 

X 


J2 


£ 


J-l 




1) 


J 


0) 




•« 
£ 


Oi 


*c3 


V 


m 




c 


LO 


o 


M 


ci 


< 


LO 


£ 




c/:' 


J 


1 '^ 





l/l 


= 


£ 


<*- 

O 


.1 f 


w 


"5 


I i 








03 1 




<v It 


S u^ 




* n 






w f 


I 2 




>, 1 


H £ 





tion, but of the part that remains in contact with the nutrient cell, only the 
stump becomes diseased. 

During the process of degeneration the myelin becomes disintegrated and 
is divided into irregular masses ; the axis-cylinder is also broken up and the 
process may finally leave nothing but a granular dibris within the sheath of 



DISEASES OF THE PERIPHERAL NERVES. 



219 



Schwann. The nuclei of the sheath undergo proliferation, and Ranvier is of 
the opinion that this is the primary factor in the entire process of degenera- 
tion. Figs. 52-55. 

This degeneration may be continued into the finest ramifications, and 
even into the muscle-plates. The cells of the endo- and peri-neurium also un- 
dergo proliferation, and the entire nerve-fibre may ultimately be changed to 
a mass of connective tissue. If degeneration has not lasted too long, regen- 
eration may set in. If the fibres have been cut, regeneration may set in if the 
cut ends are not too widely apart ; the new fibrils are offshoots from the cen- 
tral stump, and have a tendency to grow toward the peripheral stump, and 



M.TIioracic 



XSupra-Scap. 




MCircumflex. 
JtMusc.Cut 



Fig. 56. — The Brachial Plexus : the Branches involved in the Upper Arm Type of 
Paralysis are indicated by Shading. 



can grow even if decalcified bone be inserted between the cut ends (Gluck 
and others). 

In perineuritis there is swelling of the connective tissue surrounding the 
nerve-fibres, the blood-vessels are engorged, and the lymph spaces enlarged. 
The connective-tissue cells are increased. If the perineuritis last any length of 
time, an insterstitial neuritis will also be developed. This latter form is char- 
acterized by similar changes ; if the morbid process is continued, the nerve- 
fibre invariably suffers. Gombault has described a periaxillary or segmental 
neuritis in which normal segments of the nerve alternate with degenerated 
segments. In this form the myelin alone is altered and the axis-cylinder re- 
mains normal. This occurs particularly in those cases which are due to toxic 
infection. 

In diseases of the peripheral (mixed) nerves, whether there be an inflam- 
matory or degenerative process, the symptoms are. as a rule, motor and sen- 
sory in character. The paralysis is limited to the muscles supplied by the 
diseased nerve and by branches coming off below the site of injury or dis- 



220 THE NERVOUS DISEASES OF CHILDREN. 

ease. The sensory symptoms may be a hyperesthesia or an anaesthesia, ac- 
cording to the degree of change. Paresthesias are very common, and if the 
nerve is inflamed it is painful on pressure. It is a striking fact, however, 
that the motor fibres suffer much more readily than the sensory filaments ; 
possibly because the former are farther removed from the nutrient cell, and 




Fig. 57. — Dorsal Surface after Section of Ulnar Nerve. (In this and the two follow- 
ing figures the depth of the shading indicates the amount of anaesthesia. After 
Bowlby.) 

probably because a collateral innervation between sensory fibres is more easily 
established. Sensory fibres are also regenerated more easily. Further 
symptoms are, marked atrophy, loss of reflexes in the diseased member, and 
changes in electrical reaction. The degree to which the last are developed 
gives an indication of the amount of degeneration in the nerve. 



DISEASES OF THE PERIPHERAL NERVES. 



22 



Trophic disturbances are very common in connection with peripheral 
neuritis and degeneration of peripheral nerves ; but these disturbances do not, 
as a rule, appear except in severe cases. Local cyanosis, oedema, and ulcers 
are common ; the skin becomes smooth and glossy, and the growth of the 
nails may be interfered with. 




Fig. 58. — Wasting of Muscles and Areas of Anaesthesia after Section of the Ulnar 
Nerve. (After Bowlby.) 



In children, as in adults, the nerves of the brachial plexus are most fre- 
quently affected. 

The symptoms referable to lesions of the brachial plexus vary according to 
the part of the plexus involved. As in the adult, we can distinguish between 
several distinct types ; the upper-arm type, the lower-arm type, and the 
paralysis due to total plexus lesion. 



222 



THE NERVOUS DISEASES OF CHILDREN. 



In children the entire plexus may be involved, more especially in ob- 
stetrical palsies ; but often the upper arm, or Erb's type, is present. In this 
type the deltoid, biceps, brachialis, internus, and the long supinator muscle 
are affected ; at times the supinator brevis, the infraspinator, and the sub- 
scapular muscles are also involved. In Fig. 56, that part of the plexus sup- 




Fig. 59. — Palmar Surface after Section of Median Nerve. (After Bowlby.) 



plying these muscles is indicated by shading. These fibres issue from the 
fifth and sixth cerebral segments. These muscles can be excited conjointly 
if the electric pole is applied to the point discovered by Erb (Fig. 7), and 
now known by his name. The diagnosis of a partial plexus lesion can 
be made readily by testing the action of the various muscles (see tables, 
pages 18 and 22). 

Madame Klumpke has described a lower-arm type of paralysis which 
is rare in adults as well as in children ; it is due to an involvement of the 



DISEASES OF THE PERIPHERAL NERVES. 223 

nerves or root-fibres coming from the eighth cervical and first dorsal seg- 
ments. 

In this type the smallest muscles of the hands and the flexor muscles of 
the hands and the fingers are the ones chiefly involved. 

Whatever type of brachial plexus lesion may be present, the sensory dis- 
turbances are relatively slight. There is generally a diminution of tactile 
sensation, as is evidenced by the ease with which children with obstetrical 
palsies tolerate strong faradic currents. The sensory disturbance will be 
restricted to the fibres involved ; in Erb's type, for instance, sensory changes 
must be looked for in the vicinity of the shoulder and the outer surface of 
the arm. The details of sensory supply the reader will gather from Figs. 
31-34, also Figs. 57-59. 

THE OBSTETRICAL PALSIES. 

As the name implies, these palsies are due to manipula- 
tions during labor. They are very frequent in cases in 
which the physician has had to insert a finger or a hook in 
the axilla, and in those in which the arm has been severely 
pulled upon, or the arm has had to be replaced. Any 
manipulation which would press the shoulder and clavicle 
backward and upward would be liable to injure the plexus. 
In cases of breech presentation pressure upon the supra- 
clavicular spaces may cause a partial plexus paralysis. I 
have seen several cases in which this paralysis was bilateral. 
The disturbances that ensue upon these obstetrical manipu- 
lations may be the result of a simple traction upon some of 
the fibres of the brachial plexus (Erb's type most frequent), 
or of pressure upon the entire plexus. 

The clinical picture will vary according to the injury 
done. The affected arm hangs down quite limp. Although 
the child moves the fingers and the arm of the normal side, 
there is little or no power on the paralyzed side. This 
condition is the immediate result of the accident during 
labor, but as a rule it is not observed until a few davs, 
sometimes a few weeks, after birth. The affected extremity 
is a little colder than the normal one, and the muscles of 
the affected side rapidly undergo atrophy, which may for a 
time be obscured by the frequent excessive development of 
fat in children. A change of response to galvanic and 
faradic stimulation will be found according to the severity 
of the case and the age of the little patient. In the first 



224 THE NERVOUS DISEASES OF CHILDREN. 

few weeks after birth, if electrical examination is attempted, 
it will be found that the muscles of the affected extremity 
do not respond to the electric currents. At later periods, 
in the milder cases, the galvanic response of the nerves may 
be a little altered, but the muscles, as a rule, do not exhibit 
any marked change. If the case is a more severe one the 
diseased branches of the brachial plexus will fail to respond 
both to the galvanic and faradic current, and the muscles 
supplied by these branches will show either an altered 
galvanic response, the anodal closure contractions being 
greater than the cathodal closure contractions, or the af- 
fected muscles may fail to respond altogether to the inten- 
sity of current which can be employed in children. The 
ordinary tests for sensory disturbances cannot easily be ap- 
plied in very young children. There is generally some 
slight diminution in all forms of sensation, but we can only 
state with certainty that the sensations of touch and of pain 
are impaired in all but the mildest cases. In considering 
the imperfect sensation of a very young child we must bear 
in mind that, as Soltman and Westphal have shown, the 
peripheral nerves are not fully developed until several 
weeks after birth. Evidently the entire central and periph- 
eral apparatus conducting sensation is developed later than 
that transmitting motion. 

The appearance of the affected limb changes a little as 
time goes on. In some of the milder forms of obstetrical 
paralysis a complete restoration takes place within a few 
weeks, or a few months, with or without medical interfer- 
ence. In the severer forms the atrophy is apt to increase ; 
the development of the limb is retarded as compared with 
the normal side. It is common enough to have contract- 
ures at the elbows and at the wrists develop in the course 
of a few months, or after a year or more, in those cases in 
which, either from lack of treatment or from the severity of 
the injury at birth, the initial paralysis remains unaltered. 

Pathology. — It is naturally a difficult matter to deter- 
mine the exact changes which take place in any part of the 
brachial plexus in consequence of obstetrical manipulations, 
for the opportunities of a post-mortem examination are 
very rare. Roger describes a case in which the facial 



DISEASES OF THE PERIPHERAL NERVES. 225 

nerve, and the arm of the same side were paralyzed imme- 
diately after birth from pressure of the forceps. Large 
extravasations of blood were found post mortem in the 
vicinity of the stylomastoid foramen, and of the brachial 
plexus. Oppenheim, who examined one of Henoch's cases, 
discovered degenerative changes in the brachial plexus. 
In the milder forms of these peripheral lesions we must 
suppose that the disturbance of function was due to slight 
mechanical injuries, to a stretching or tearing of some of 
the fibres, possibly to slight injury of the nerve-sheath, or 
a mild form of inflammatory reaction in the nerve-tissue. 
In the severer cases the lesion may amount to an actual 
tear, to a severance of the nerve-fibres, or, in the case of 
subluxation of the head of the humerus, to actual compres- 
sion, with loss of function, of the nerve-fibres. Every pos- 
sible degree of peripheral neuritis may therefore be the re- 
sult of these obstetrical lesions. 

Diagnosis. — The diagnosis of obstetrical palsies is one 
that demands a most careful examination of the paralyzed 
member. The differential diagnosis is to be made between 
these obstetrical palsies and the so-called " birth palsies " 
due to cerebral lesions. 



Peripheral Palsies (Obstetri- Cerebral Birth Palsies, 

cal Palsies of Brachial 
Plexus). 

i. Arm only affected. I. Hemiplegia or diplegia common ; 

brachial monoplegia rare. 

2. Flaccid paralysis with atrophy. 2. Spastic paralysis, with or without 

atrophy, with tendency to rigid- 
ity. 

3. Deep reflexes absent, surely not 3. Deep reflexes increased. 

exaggerated. 

4. Changes in electrical reaction from 4. No changes in electrical reaction. 

simple loss of faradic response to 
complete reaction of degenera- 
tion. 

5. No convulsions. 5. Convulsions apt to occur and to 

be repeated. 

6. Deformity and arrested growth of 6. Flexion contraction of fingers. 

entire extremity. wrist, and elbow. 

7. Sensation may be impaired. 7. Sensation not affected. 

T 5 



226 THE NERVOUS DISEASES OF CHILD REX. 

There is no other condition from which these obstetrical 
palsies need to be differentiated if the child is examined 
within a reasonable time after birth ; but if, as is so often 
the case, the child is brought to a physician when it has 
reached the age of six, seven, or even twelve years of age, 
the true cause of the palsy may be difficult to fathom. At 
my clinic I often see cases for which nothing has been 
done, and the cause is elicited only after the closest in- 
quiry from the mother. In older children it is a question 
whether the palsy dates from birth, or whether some acci- 
dent in the first few years of life may not have been the 
cause. The pulling of the arm in some of the games the 
children play, wrenching and twisting it as they do with 
considerable force, may result in palsies very much like 
obstetrical palsies. Poliomyelitis is not easily to be con- 
founded with these obstetrical palsies, for the former is 
rare in the first year of life, and relatively rare in the upper 
extremities ; the atrophy is generally more extreme, sensa- 
tion is not involved, and complete recovery is not common 
in the spinal paralysis of children. 

Prognosis. — If properly cared for, recovery may be ex- 
pected in all but the most severe forms of obstetrical pe- 
ripheral palsy. According to the severity of the symptoms, 
the length of time that it will take before recovery sets in 
can also be foretold, with some degree of certainty, and 
for this purpose the electrical behavior of the nerves and 
muscles constitutes a fair test for the amount of damage that 
has been done. Even though the paralysis be complete, if 
the faradic response of the branches supplying the affected 
muscles is not entirely lost, the probability is that complete 
recovery will set in within a period of two to three months. 
If the faradic response is lost, but the galvanic formula is 
not altered, the restoration of function may be expected 
within a period of six months ; but if both the faradic and 
the galvanic responses are entirely gone or seriously im- 
paired, it may be a year, or even two years, before the arm 
can be properly used. In the severer cases, in which a 
great deal of injury has been done to the brachial plexus, 
it will not be well to promise much regarding the time or 
the possibility of complete recover)'. Some of these cases 



DISEASES OF THE PERIPHERAL NERVES. 227 

are extremely stubborn ; this is to be emphasized, for the 
majority of authors seem to regard these conditions too 
favorably. Much will depend upon the accuracy of treat- 
ment. One point which I wish to impress upon the reader, 
however, is that in a fair proportion of all these cases the 
physician may reassure the parents regarding the future 
condition of the paralyzed extremity. 

Treatment. — If the child is seen immediately after 
birth, and the diagnosis has been properly made, the best 
the physician can do is to let the limb severely alone for a 
period of at least two weeks ; at the end of that time the 
exact damage that has been done can be properly estimated 
and the necessary therapeutic measures can be employed. 
Wrapping up the arm carefully, so as to avoid any further 
injury to it, and allowing it to be bathed as the rest of the 
body is, will be all that is necessary. After a period of 
two weeks I would advise the use of light friction and 
massage, so as to keep up the nutrition of the muscles, and 
I am also in favor of directing an intelligent nurse or rela- 
tive to move the parts systematically so as to overcome 
any tendency to contracture. After a period of four to 
six weeks electricity may be employed simply as a mode of 
exercise. If the parts respond to a mild faradic current 
such may be employed, but if the faradic current fails to 
elicit a response it is sheer waste of time to employ this 
form of electricity, and it will be better to use a mild gal- 
vanic current, using that pole which will produce contrac- 
tions. Sittings of from five to ten minutes every day, or 
every other day, are quite sufficient, and will be of some 
benefit in the way of preventing excessive atrophy and of 
preventing the formation of contractures. 

As soon as there is a tendency to the development of 
contractures, it will be well to place the arm in a splint 
in such a way as to oppose the contracture by simple me- 
chanical force, and to take the arm out of the splint dailv 
for the purpose of treating it by proper massage and elec- 
tricity. If deformities have arisen as the natural result of 
disease, or from neglect, the child should be placed in the 
hands of an orthopedic surgeon and an attempt should be 
made by every known surgical procedure to correct the 



228 THE NERVOUS DISEASES OF CHILDREN. 

existing trouble. The old belief that a child will outgrow 
such deformities is absolutely unfounded. It is an unfort- 
unate fact, but true, nevertheless, that children outgrow 
only the very mildest disturbances of the nervous system, 
which is equivalent to saying that some troubles undergo 
spontaneous cure. 



Paralysis of the lower limbs due to obstetrical manipulations is reported 
every now and then, but it is extremely rare. The lumbar and sacral plexuses 
are so well protected at every point of emergence from the spine and in their 
course through the gluteal and pelvic regions that they are not easily disturbed 
by any amount of manipulation or traction upon the legs. An injury of these 
parts is, however, conceivable. If present, the paralysis will resemble the 
paralysis of the adult in the number of muscles involved and in the character 
of the palsy. It is interesting in this connection to note that Ross, some 
years ago, supposed that traction of the legs was occasionally responsible for 
spastic paraplegia in children. He does not refer to the effect upon the 
nerve-roots, but supposes that such traction would be followed by derange- 
ment of the fibres in the spinal cord itself. This view has not been accepted, 
and it has been shown that cases of spastic paraplegia due to traumatism 
during birth are due, in the vast majority of cases, to cerebral and not to 
spinal injuries. 

Still another obstetrical palsy occurring quite exceptionally is facial paral- 
ysis due to compression of the peripheral portion of the nerve by the blade of 
the forceps. This is an unusual occurrence, for if the blades of the forceps 
are properly applied they should not be in the vicinity of the facial nerve ; 
but accidents will happen and the blades occasionally slip even if applied by 
skilful obstetricians, and thus cases of obstetrical facial nerve palsy are not 
entirely unknown. This may be the result of actual pressure, and in other 
cases an extravasation of blood into the region of the parotid is likely to com- 
press the nerve sufficiently to cause temporary palsy. Such a palsy bears all 
the evidences of peripheral facial palsy, in contradistinction to the facial palsy 
that is associated with hemiplegia. (See page 234.) 

The prognosis in these cases is, on the whole, favorable. In a few cases, 
however, a degeneration of the nerve occurs in consequence of the severity 
of the compression, and under these circumstances permanent disfigure- 
ment of the face may be the result. Henoch reports two such cases. Par- 
rot and Troisier have examined such nerves post mortem. I have the rec- 
ords of two cases, one in a girl of twelve, the other in a woman of some 
twenty-two years of age, in whom facial palsy with unequal development of 
the two halves of the face is the result of this obstetrical accident. 

The treatment of these cases is exactly the same as that for all other pe- 
ripheral palsies. Light massage and the use of the electric current are the 
only measures that can be safely recommended. In the use of the current, 
which I would not advise applying until the child is at least four weeks old, 



DISEASES OF THE PERIPHERAL NERVES. 



229 



great care should be exercised not to give too strong a current to the head 
nor to cause any sudden interruptions of the current, for the effect upon the 
brain would be decidedly disagreeable. Medication by drugs is not in order. 



FACIAL PALSY (BELL'S PARALYSIS). 

Disease of the seventh nerve is not infrequent in children 
from the same causes that result in facial paralysis in the 
adult. We have to con- 
sider, first, rheumatic fa- 
cial palsy, more properly 
perhaps refrigeration pal- 
sy ; second, facial palsy 
due to ear disease ; and 
third, facial palsy as an 
accompaniment of disease 
at the base of the brain. 

Rheumatic facial pal- 
sy may occur in children 
at almost any age. Al- 
though I have seen it in 
a child nine months old, 
it is rare before the age 
of three years, and is most 
frequent between the ages 
of six and fifteen years. 
Sudden draughts of cold 
air are by far the most 

frequent Cause. The Side FlG - 60. -Diagrammatic figure showing the Re- 
lations of the Seventh and Glosso-Pharyngeal 
Nerves and the Course of the Taste Fibres. 
The lines I, 2, 3, 4 mark off different segments 
of the nerve. N.f., facial nerve ; N. a. , Acous- 
tic nerve; G. ph., glossopharyngeal nerve; 
G. g., geniculate ganglion ; N. p. s., great su- 
perficial petrosal from fifth nerve ; vV. c, c.p. t., 
tympanic plexus ; the dotted line vS". /. s. indi- 
cates salivary fibres ; the other dotted line in- 
dicates taste fibres ; C/i. /., chorda tympani ; 
N. st. , stapedius nerve; F. st, stylo-mastoid 
foramen. (Modified from Erb by Dana.) 




of the face exposed to the 
draught is generally the 
one that is paralyzed. In 
a few instances it is diffi- 
cult to make out the ex- 
act cause, but even under 
such circumstances re- 
frigeration is very prob- 
able. I am not aware that 
facial palsy occurs in connection with the various diatheses 
in children as it does in the adult, where it is met with in 
association with diabetes or tuberculosis. 



230 THE NERVOUS DISEASES OF CHILDREN. 

The symptoms of facial palsy will be readily under- 
stood by an examination of Fig. 60. The distal portion (1) 
is the one which is generally affected in these rheumatic 
cases. All the branches of the facial nerve are involved, 
but there is no interference with taste, with hearing, with 
movement of the palate, or with the secretion of saliva. 
The paralysis involves the muscles of the eyes, the nose, 
the cheeks, and the lips. On inspection of the child's face it 
is easy to make out which side is affected, as the eyelids of 
the diseased side are wide open (lagophthalmos). If the 
child is asked to close the eyes it does so with ease on the 
sound side, but the diseased side fails to respond.* In 
this effort there is over-action of the frontal muscles and 
of the corrugator supercilii on the sound side ; the diseased 
side showing either diminished action or a total paralysis. 
The child cannot pull up its nose ; in bloAving out the cheeks 
the diseased side is flabbier than the sound side, and if the 
attempt is made to whistle or to pout the lips, as in kissing, 
the insufficient action of the muscles on the diseased side 
becomes very evident. If asked to show the teeth, the 
muscles of the sound side overact, and the mouth is drawn 
toward the healthy side. By this movement the paralysis 
is often revealed, even if the parts appear perfectly normal 
while at rest. The tongue when protruded deviates toward 
the sound side ; this deviation was formerly considered to 
be apparent, not real ; but Hitzig has shown that there is 
an actual deviation toward the sound side, and supposes 
that this is due to the fact that the tongue wishes to avoid 
contact with the corner of the mouth on the paralyzed side. 
Contractures of the muscles of the paralyzed side occur in 
the later stages of a facial palsy. The lower branches re- 
cover, as a rule, more slowly than the upper, and in several 
cases I have observed that the lower and middle muscles 
could be contracted only if the attempt was made to close 
the eyes (Fig. 61). 

Sensory disturbances are entirely absent, as the facial 
nerve is a purely motor nerve, the sensory fibres of the face 

* A few cases of double facial palsy have been reported (Mott, Huebschman) ; a suc- 
cessive involvement of the two sides is more frequent. The author has observed it in 
cases associated with diabetes in the adult. 



DISEASES OF THE PERIPHERAL NERVES. 



231 



coming from the fifth nerve. I have seen a few cases of 
severe neuralgic pains complicating facial palsy in children, 
but to account for it there has always been some additional 
cause, as in a boy of fourteen, whom I recently examined, 
in whom decayed upper teeth offered the explanation for 
the neuralgic supraorbital pain. 

Whatever part of the nerve is involved, provided it be 
between the nucleus of the facial nerve in the pons and 
the periphery, electrical changes will be found. In all but 




Fig. 61. — Boy with Facial Palsy, in Stage of Recovery. Slight contracture of para- 
lyzed side (B) ; in figure A mouth is pulled to the paralyzed side when boy attempts 
forcibly to close left eye ; also deepening of naso-labial fold. 

the very mildest cases the faradic response of the nerve 
as well as of the muscles is lost. My own experience has 
proved, however, that there is an exception to this rule, and 
that is, that if the patient be examined within the first forty- 
eight or seventy-two hours after the onset of the palsy, the 
faradic response may still be present ; it rapidly diminishes, 
however, and after a period of three days, in the vast ma- 
jority of cases, it is entirely lost. The galvanic response 
of the nerve may be increased during the first few da vs. 
but it is soon diminished or lost. The galvanic irritability 
of the muscles supplied by the facial nerve shows many in- 
teresting changes. In the earlier stages of the disease, and 



232 THE NERVOUS DISEASES OE CHILDREN. 

in all but the severest cases, there is an increased galvanic 
excitability for a period of about two weeks. The same 
currents which produce very strong contractions on the 
diseased side give weaker contractions on the sound side. 
This can be easily demonstrated in the case of the orbicu- 
laris oris. If a very weak current be applied to the sound 
half of the muscle, contractions may be seen in the diseased 
half and not in the normal half, the increased excitability 
in the latter being so great that the small amount of current 
diffusing to the paralyzed side is sufficient to produce a 
contraction, while the full current is not sufficient to make 
the healthy half contract. During this period the cathodal 
closure contraction of the diseased muscle is generally 
greater than its anodal closure contraction. After a little 
while the increased excitability diminishes and soon falls 
below that of normal muscular tissue. The polar con- 
tractions also indicate a change, the cathodal closure con- 
traction first being equal to the anodal closure contraction ; 
later on in the severer cases the order is entirely reversed. 

As the disease approaches the period of recovery the 
galvanic formula approaches more nearly to the normal, 
the cathodal closure contractions being equal to the anodal 
closure contractions. Finally, the cathodal closure con- 
traction becomes greater than the anodal contraction, and 
the contractions that have been sluggish once more become 
prompt and short. The faradic response is, as a rule, rather 
longer than this in returning, and it is not uncommon to 
see the full power of all the muscles return before the nor- 
mal electrical reactions have been established. In the case 
of a boy (Fig. 61) the faradic response of the muscles and 
a normal galvanic formula were not restored until several 
months after the contractures had set in, and after most of 
the muscles could be innervated tolerably well. 

In facial palsy due to ear disease, the symptoms can 
be easily accounted for by the position of the nerve in the 
Fallopian canal. The nerve may be involved during 
an attack of otitis media, and as this ear affection occurs 
often enough in children in association with several 
acute infectious diseases, notably scarlatina, this form of 
facial palsy is quite a frequent occurrence. It does not 



DISEASES OF THE PERIPHERAL NERVES. 233 

occur as readily in the earlier stages of an otitis as in the 
later suppurative stages, when, in consequence of caries of 
the bone, the facial nerve is directly involved. According 
to the severity of the disease the paralysis will be more or 
less complete, and as far as the facial nerve is concerned the 
symptoms will resemble those of the rheumatic form, but 
naturally the association of facial palsy with defective hear- 
ing or the persistence of a purulent discharge from the ear 
will point to the actual cause of the paralysis. 

I have seen facial palsy occur also after mastoid opera- 
tions for ear disease. In these cases an accident during the 
operation is often sufficient to break through the fine bony 
plate separating the facial nerve from the ear structures, 
and a more or less severe facial palsy is the result. This 
occurred in a little patient whose case I reported a few 
years ago, in whom the mastoid operation was done for the 
relief of epileptic attacks which were caused by the reten- 
tion of pus. On his recovery from the operation a distinct 
facial palsy on the side of the operation was noticed, and 
existed for a period of very nearly three months, after 
which complete recovery took place. 

As in the adult so in the child, paralysis of the face may 
accompany lesions at the base of the brain and in the pons. 
If the lesion is at the base, other cranial nerves are involved 
as well as the facial, and the diagnosis can therefore be 
readily made. It is common enough to find the facial 
nerve partially or totally paralyzed in cases of basilar men- 
ingitis, whether tubercular or non-tubercular. In cases of 
injuries to the skull, or in tumors occupying the middle or 
posterior fossa, facial palsy is one of a large series of symp- 
toms and has but little diagnostic value excepting in its as- 
sociation with affections of other cranial nerves. It may be 
stated in this connection that in cases of cerebellar tumor, 
which are by no means rare in children, both the acoustic 
and the facial nerves, which lie in very close juxtaposition 
to each other and are easily pressed upon by a cerebellar 
growth, are often paralyzed and give rise to symptoms 
which are almost pathognomonic of cerebellar disease. If 
the lesion is in the pons, and particularly if it be below the 
decussation of the facial nerves, paralysis of the face is 



234 THE NERVOUS DISEASES OF CHILDREN". 

associated with alternate hemiplegia. In these cases every 
branch of the facial nerve is apt to be involved. If the 
lesion be a neoplasm other symptoms pointing to compres- 
sion of the cranial nerves and indicating the presence of 
tumor within the cranial cavity will naturally be associated 
with those mentioned before. 

The diagnosis of facial palsy is easy to make, and the only difficulty that 
arises at times is to determine whether the palsy is indicative of a central or 
a peripheral lesion ; but this difficulty can be readily met by keeping in mind 
the fact that if the lesion is in the brain anywhere above the pons the upper 
branches of the facial escape altogether and the electrical responses remain 
entirely normal ; furthermore in such a case the facial palsy is associated with 
hemiplegia on the same side of the body as is the paralysis of the face. It is 
far more difficult to determine the exact part of the nerve that is diseased. 
As the intensity of the affection depends somewhat upon the extent of the 
nerve tract that is diseased, this inquiry into the accurate localization of the 
disease has a practical as well as a scientific interest. Taking the diagram 
on page 229 we can study the variation of symptoms according to the part 
that is affected. If the disease is limited to that part outside of the Fallo- 
pian canal, there is complete paralysis of every branch of the facial nerve 
with the exception of the posterior auricular. Reflexes are wanting, but there 
are no disturbances of taste or hearing, no paralysis of the palate, and no dis- 
turbances of salivary secretion. If the disease happens to affect the part of 
the facial nerve as far as, but not including, the chorda tympani (Fig. 60 line 
2), the symptoms are paralysis of all the branches of the facial, including 
the posterior auricular. 

The same symptoms as the above, with the addition of a disturbance of 
taste in the anterior portion of the tongue and a diminution in salivary secre- 
tion points to an involvement of the facial nerve from the point of junction 
of the chorda tympani to the junction of the stapedius with the main trunk 
(from 2 to 3). 

If that part of the nerve lying between the stapedius and the ganglion 
geniculi is diseased, the symptoms, in addition to total paralysis, are changes 
in taste, diminution in salivary secretion, and abnormal acuity of hearing, but 
there is no paralysis of the soft palate. The above symptoms, with the addi- 
tion of paralysis of the soft palate, point to a lesion in the region of the gen- 
iculate ganglion (division 4 to 5) ; but on this point there is much doubt. 
See table, p. 13. 

Paralysis of all the peripheral branches, a*s well as paralysis of the soft 
palate, abnormal acuity of hearing, decreased salivary secretion, but no involve- 
ment of taste, help to locate the lesion above the geniculate ganglion, and be- 
tween it and the exit of the facial nerve from the brain (division 5 to 6). If this 
part of the facial tract is diseased other cranial nerves are frequently affected. 
In such cases we are apt to have paralysis of the abducens, loss of hearing on 
the same side as the facial palsy, tinnitus aurium, and the reaction of degen- 



DISEASES OF THE PERIPHERAL NERVES. 235 

eration of the acoustic nerve. If we have total palsy of every branch, palsy 
of the soft palate without disturbance of taste and with simple diminution of 
electrical excitability, without any other marked symptoms, we may locate 
the lesion in the facial nucleus ; but this form of palsy is recognized more 
definitely if other cranial nerves are simultaneously affected, such as the 
hypoglossal, the spinal accessory, the vagus, the trigeminal nerve, etc. 

The association of the symptoms just mentioned with paralysis of the arm 
and leg of the opposite side, refer the lesion to the pons. If there is but par- 
tial paralysis of the face, if the electrical excitability is not affected, if the re- 
flexes are normal, and if the extremities of the same side are affected, the 
lesion is in the crus or in the hemispheres, say in the internal capsule. If the 
facial nerve and the oculo-motor nerve of opposite sides happen to be affected 
the lesion is positively in the crus on the same side as the ocular palsy. 

The course and prognosis of all forms of facial palsies 
will depend entirely upon the nature of the morbid proc- 
ess. If the disease is due to caries of the petrous por- 
tion of the temporal bone, to malignant tumor, to fracture, 
to tubercular meningitis, or to any form of meningitis, the 
prognosis is extremely grave. Even those cases depending 
upon specific diseases at the base are not as amenable to 
treatment as one might expect. Those due to parotitis or 
to internal otitis recover if the disease itself is not of the 
destructive type. Cases due to simple section of the 
nerve, as in cases of operation, yield a tolerably favorable 
result. 

The prognosis of the rheumatic forms of facial palsy 
varies according to the intensity of the disease, but it does 
not depend apparently upon the amount of nerve-tissue 
that is affected, for, on the whole, cases in which the nerve 
within the canal is involved take about as favorable a 
course as do those in which the pes anserinus is the only 
part affected. Erb distinguishes three forms — a mild form, 
a medium form, and a severe form of facial paralvsis. His 
division depends entirely upon the electrical conditions 
present at the end of the first week. I cannot agree alto- 
gether with his statement that if the electrical excitability 
is entirely normal at the end of the first week, as in light 
forms, recovery will set in in two or three weeks. If at 
this same period the faradic and galvanic excitability are 
slightly diminished recovery may be expected in from four 
to six weeks, and if at the end of the first week the elec- 



236 THE NERVOUS DISEASES OF CHILDREN. 

trical excitability of the nerve is very much diminished or 
totally lost, the disease will run a course of many months. 
I have seen cases get well practically within four weeks, in 
which the faradic and galvanic excitability of the nerve 
was diminished very much from the very start, and I 
should be inclined to formulate the following statements : 

I. If at the end of the first week, or, still better, at the 
end of the second week, the nerve responds at all to the 
faradic or galvanic current, a prompt recovery in about 
four weeks may be expected. 

II. If at about the same time the nerve fails to respond, 
but the muscles show a diminished or altered galvanic re- 
sponse, the disease is likely to run a course anywhere be- 
tween one and three months. 

III. If the muscles respond but feebly to strong cur- 
rents, if the galvanic formula is altered, and if the contrac- 
tions are extremely slow, the disease may run a course any- 
where between six months and a year, or even longer. If 
after a period of two months no electrical reaction can be 
observed, the degeneration is very complete, and a paral- 
ysis lasting at least a year, if not longer, may safely be pre- 
dicted. 

Treatment. — We need say little of the treatment in cases 
in which the facial palsy is only one of many symptoms 
pointing to gross cerebral disease. Under such circum- 
stances treatment directed to the relief, or possibly the cure, 
of these conditions will have to be employed. In the cases 
of facial palsy due to ear disease, proper surgical treatment 
should be recommended at a very early day. Much doubt 
has been expressed as to whether any treatment can cur- 
tail or cure the rheumatic palsies. The milder forms will 
undoubtedly get well even if no treatment is attempted. 
In the more chronic cases the use of the electrical current, 
particularly of the galvanic, can be safely recommended, 
for I have the distinct impression that cases so treated, and 
particularly those that have been properly treated from the 
very start, run a somewhat shorter course than those in 
which no electrical treatment has been attempted. 

Remak's recent article gives statistical evidence in favor 
of this view, and puts a quietus for the present upon those 



DISEASES OF THE PERIPHERAL NERVES. 237 

who think that there is nothing in electro-therapeutics but 
hypnotism and suggestion. 

The electrical current, even if it have but slight influ- 
ence in restoring the power of conduction in the diseased 
nerve, is surely of value, as it is in paralysis of the extremi- 
ties, in exercising parts that cannot be moved by the will, 
and for this reason alone it deserves to be employed. 

Counter-irritation has been repeatedly tried. This may 
be accomplished by blistering either with emplastrum can- 
tharidum just in front of the ear or over the mastoid, or by 
the use of the actual cautery. I am free to confess, however, 
that the value of counter-irritation seems questionable in all 
cases, and particularly in those in which counter-irritation is 
not indicated to allay pain that is so frequently associated 
with neuritis. The application of leeches is to be thoroughly 
condemned, except possibly in those cases in which the fa- 
cial palsy is associated with ear disease. This practically 
limits us to the use of electricity as the only therapeutic 
measure which promises some relief in the severer cases. 
In these cases the faradic current, as recommended by 
Erb and Duchenne, may be employed for the first week 
or two, but after that a stabile galvanic current applied 
over the mastoid and along the peripheral divisions of 
the facial nerve is more in order. As soon as the ex- 
citability of the nerves and muscle is increased, or shows 
an approach to the normal, regular electrizations of the 
paralyzed muscles, in sittings of ten minutes or more, two 
or three times a week, should be practised. I wish to cau- 
tion the physician, however, against the use of strong cur- 
rents or against the use of the electrical current without 
the safeguard of a galvanometer. A current varying be- 
tween two and four milliamperes is quite sufficient for all 
purposes. I have also directed my patients, as soon as the 
least power returns, to practise contraction of the muscles 
(closing of the eyes, pouting of the lips, etc."), while look- 
ing into a mirror. 

As for medicinal measures, they may be discarded alto- 
gether. In many cases I have tried strychnia both per os 
and in the form of hypodermic injections, and I am firmly 
convinced that the effect of such medication is absolutely 



238 THE NERVOUS DISEASES OF CHILDREN. 

nil, nor can we expect much more from the use of irritat- 
ing ointments or douches to the face. If drugs must be 
administered it is far better to give the ordinary tonics 
which will help to build up the general condition of the 
child ; and with this end in view, iron, arsenic, cod-liver oil, 
will be of far more service than any other remedies. 

Other Peripheral Palsies. — Almost any peripheral palsy which is 
known to occur in the adult may at times occur in the child. Of these I will 
make special mention only of a peripheral palsy, generally of traumatic ori- 
gin, affecting the peroneal nerve. Injury to this nerve occurs readily enough 
if a child happens to be violently struck upon the leg, or if the leg happens 
to be squeezed against a post, a chair, or, as in one case I have seen, against 
the edge of the bed. I mention this special palsy because it may lead to 
slight difficulties in diagnosis, inasmuch as confusion may arise with acute 
anterior poliomyelitis which affects the muscles supplied by the peroneal 
more often than those supplied by any other nerve. 

The onset of the paralysis without fever, the presence of considerable 
pain, the marked and rapid tendency to recover} 7 , and the history of a trau- 
matic injury will help to distinguish this peripheral palsy from the essential 
paralysis of children. The course, prognosis, and treatment of such a pero- 
neal neuritis will be exactly the same as in the case of other nerve palsies. 

Spasm of various peripheral nerves is common in young persons. The 
facial and the spinal accessory nerves are frequently affected ; the trigeminal 
in rare instances only. Spasm of the facial nerve (tic convulsif) is much 
less frequent than in adults ; a spasm involving an entire facial nerve of a 
child is very rare indeed ; but partial spasms are observed in the form of nic- 
titation (clonic spasm) or of blepharospasm (tonic spasm) of the obicularis 
palpebrarum. These movements are generally due to the influence of cold, 
to irritation of the trigeminus, but are also frequently of psychic origin. In 
the cases of clonic spasm of the entire nerve, all sorts of extravagant grimaces 
are indulged in. These spasms must not be confounded with habit chorea. 

Spasm in the distribution of the spinal accessory nerve involves either the 
sternocleido-mastoid or the trapezius (spasmodic wryneck). 

The spasm may be reflex in origin, or due to irritation of the nerve in its 
course from the medulla and spinal cord to the periphery. The position and 
movements of the head will vary according to the muscles involved. These 
spasms may be unilateral or bilateral. 

If we remember that spasm, whether clonic or tonic, denotes an excess of 
normal function, the symptoms can be easily understood by reference to the 
tables on pages 15 and 16. 

Ordinary wryneck, so frequent in children, is due to a rheumatic myo- 
sitis. Symptomatic wryneck occurs in connection with cervical adenitis, 
abscesses and caries of the cervical spine. Congenital wryneck is due to 
some pre-natal disturbance ; it may be of intra-uterine origin, and denote im- 
perfect development, in which case it is generally associated with atrophy 



DISEASES OF THE PERIPHERAL NERVES. 239 

of one-half of the face ; at times it is due to obstetrical injuries in cases of 
breech and foot presentations. 

The treatment of spasms of the peripheral nerves consists in the use of sed- 
atives, such as opium, conium, codein, chloral, and bromides ; in some cases 
the valerianate of zinc, or the ammoniated tincture of valerian, might be given. 
The galvanic current may be applied in the milder cases. The attempt may 
be made to check the spasms by hypodermic injections of hydrobromate of 
hyoscine, one two-hundredth to one one-hundredth grain, or by the use of 
atropia (one-fiftieth to one-tenth grain, as recommended by Leszynsky). 

Tenotomies are in order in the congenital and chronic cases. Keen has 
suggested resection of the posterior branches of the upper three or four 
cervical roots ; but the advisability of this procedure is still in doubt. 

Spasm of the hypoglossal nerve I have not seen in children. The com- 
plex co-ordinated spasms have been considered in Chapter VI. 



BIBLIOGRAPHY. 

PERIPHERAL PALSIES. 

Bernhardt, M. : Arch. f. kl. Med. vol. xiv., 1874, p. 433; Zeitschr. kl. Med., 
1882, p. 415 ; Neurologisches Centralblatt, 1886, p. 141. 

Erb : Diseases of the Peripheral Cerebro-spinal Nerves, in Ziemssen's Cy- 
clopaedia. (Contains full literature to 1876.) 

Hirt : Lehrbuch ; Second ed., p. 364. (Contains ample references.) 

Hitzig : Berliner klinische Wochenschrift, 1892, p. 1270. (Position of 
tongue in facial palsy.) 

Hoffman : Zeitschrift f. Nervenheilkunde, vol. v., p. 72. 

Huebschman : Neurologisches Centralblatt, November, 1894. 

Klumpke : Revue de Medecine, July, September, 1885. 

Leszynsky : Journal of Nervous and Mental Disease, January, 1890. 

Mott : British Medical Journal, December 2, 1893. 

Oppenheim and Siemerling : Arch. f. Psych., 1887. 

Parrot et Troisier : (Facial Paralysis in the New-born), quoted by Henoch, 
p. 227. 

Pfeffer: Klumpke 'sche Lahmung., Deutsche Zeitschr. f. Nervenheilkunde, 
1 891. 

Remak : Zeitschrift f. Nervenheilkunde, vol. iv., p. 377. 

Roger: Journal fiir Kinderkrankeiten, 1864 (quoted after Henoch). 

Ross and Bury: On Peripheral Neuritis. London, 1893. 

Van Bibber : In Keating 's Cyclopaedia, page 788. 



CHAPTER XIII. 

MULTIPLE NEURITIS. 

Neurology has few more signal achievements to show 
than the advances that have been made in the study of 
multiple neuritis within the past decade. Many diseases 
which were formerly supposed to be due to an affection 
of the spinal cord, and which were often confounded with 
poliomyelitis, and with various forms of acute and sub- 
acute myelitis are now positively known to be due to poly- 
neuritis. The term multiple neuritis or polyneuritis is 
used to embrace all those diseases in which the symptoms 
are due to disease of several or many of the nerves of the 
body. There is not only a tendency to multiplicity but 
also a tendency to symmetrical development. 

Many of the older writers recognized the possibility of multiple nerve af- 
fections, and a multiple neuritis, as such, was recognized by Dusmenil as far 
back as 1 864, and even a few years earlier Virchow had described a multiple 
neuritis occurring together with leprosy. Going back still further, the histor- 
ical investigations have led to the discovery that Dr. Jackson, of Boston, had 
recognized peripheral multiple neuritis as early as 1822 ; but all these re- 
searches, even including those of Duchenne, did not help to place the knowl- 
edge of multiple neuritis upon a firm basis. The subject was revived by Jof- 
froy, iri 1879, an d Leyden, in 1880, and since that time a legion of articles 
have appeared by writers of almost every nationality. 

Comparatively few writers, however, have had much to say regarding 
multiple neuritis occurring in children. Diphtheritic paralysis, to be sure, was 
discussed clinically long before it was known that it was due to peripheral 
neuritis. Multiple neuritis is not so frequent in children as in adults, for the 
simple reason that the causes leading to it are not as powerful in early life as 
later on ; and yet cases are common enough to necessitate a discussion of 
them in this book. 

Symptoms. — Whatever the cause of the multiple neuritis 
may be the symptoms are, with few modifications, practi- 



MULTIPLE NEURITIS. 



241 



cally the same in all cases. Though the development of the 
various symptoms is subject to great variations, the symp- 
toms are such as we might well expect on physiological 
grounds from disease of the peripheral mixed nerves. The 




Fig. 62. — Young Boy with Multiple Neuritis, showing Double "Wrist Drop" and 

Slight " Foot Drop." 

most characteristic feature of multiple neuritis is the asso- 
ciation of motor paralysis with sensory paralysis, the dis- 
tribution of each harmonizing with the other and showing- 
very definite anatomical limits. The paralysis is of the 
flaccid order, leads at an early date to atrophy of the at- 
16 



242 THE NERVOUS DISEASES OF CHILDREN. 

fected muscles, and the electrical conditions are altered so 
that Ave may find almost every possible form of the reac- 
tion of degeneration from a mere loss of faradic irritabil- 
ity to an absolute loss of galvanic response on the part of 
the nerves and muscles. The distribution of the paralysis 
is, as a rule, entirely symmetrical, and may affect either 
the upper or lower, or all four extremities ; it may involve 
every part of all the extremities, and is the one affection 
which, perhaps more frequently than any other, leads to 
a complete paralysis of every limb of the body. It is one 
of the diseases, therefore, which the physician should bear 
in mind if he finds a patient who is totally paralyzed with 
the bare exception of the head and tongue, the jaws and 
eyes ; sometimes some of the latter parts may be paralyzed 
as well. Certain groups of muscles are more readily af- 
fected than others ; thus we find that the extensors of the 
wrist and the extensors of the feet are very frequently in- 
volved, and at an early period of the disease " wrist-drop " 
and " foot-drop " are characteristic phenomena of the dis- 
ease. With the paralysis sensory symptoms go hand in 
hand ; paralysis associated with pain may be noted in some 
cases of spinal-cord disease, as in the acute stages of a 
poliomyelitis, but the persistence of pain in those parts 
which are paralyzed, and along the nerve-tracts, points very 
strongly to a multiple neuritis. Whenever we suspect that 
a paralysis or a deformity may be the result of a multiple 
neuritis, the previous occurrence of a painful affection and 
the duration of the same must be determined with the 
greatest care. If the patient is seen during the acute stage of 
the disease the tracts of the peripheral nerves will be found 
painful, and this objective proof will be added to the sub- 
jective symptoms which the patient describes. But the ab- 
sence of pain, as evidenced by cases reported by Barrs, 
need not militate against the diagnosis. 

The motor and sensory paralysis is not developed as rapidly as is the 
case in central affections. There is a premonitory period during which 
numbness, tingling, sensations of heat or cold, in the parts to be affected 
later on, are the annoying symptoms, and after a premonitory period that 
may vary from a few days to a few months the development of the paralysis 
may be acute, subacute, or chronic. In the majority of the cases it is sub- 



MULTIPLE NEURITIS. 243 

acute or chronic, and it often requires a period varying between one and two 
weeks until the weakness of the parts has advanced far enough to be con- 
sidered an actual paralysis. The onset is marked occasionally by the oc- 
currence of fever, and if the disease is due to any toxic agent that has con- 
stitutional effects the beginning may be signalized by convulsions as well as 
by rigors. Under the latter circumstances the symptoms may very closely 
resemble those of an acute poliomyelitis, but it is rare, after all, to find as 
stormy an onset in cases of multiple neuritis as in spinal infantile paralysis. 

Before the paralysis becomes absolute, if it ever reaches 
that stage, tremor of the parts and inco-ordination may be 
associated with loss of power and loss of sensation. The 
patient may be able to stand or walk, but does so imper- 
fectly, sways with the body when attempting to stand, and 
may fall if the eyes are closed. The reflexes connected 
with the parts affected by the paralysis are almost inva- 
riably diminished or lost ; this is particularly true of the 
knee-jerk, since the trouble so often begins in and involves 
the legs to a greater extent than any other part of the 
body. The reflex is often absent before the paralysis is 
developed. An exception to the rule of loss of knee-jerk 
has been reported by Striimpell. A few years ago he de- 
scribed a case in which the reflexes were increased, and 
this increase he attributed to irritation of the afferent por- 
tion of the reflex arc. 

The more distal parts of the extremities are, as a rule, 
the first to be affected. In the lower extremities the ear- 
lier stages of the disease are marked by an inability to 
raise the toes while the heel remains on the ground, show- 
ing a weakness of the extensors of the toes, and implying 
a loss of function of the branches of the anterior tibial 
nerve. It is a curious fact that the muscles supplied bv 
the anterior tibial nerves are not only most frequentlv af- 
fected in multiple neuritis, but they also innervate the re- 
gion which is frequently involved in poliomyelitis. In the 
upper extremities the extensors in the forearm are affected 
in the earliest stages of the disease, and from these parts 
the paralysis may extend upward in both extremities until 
the greater part of the muscles are affected or until total 
paralysis of all the extremities is established. The thenar 
and hypothenar groups are hot affected at the outset. It 



244 THE NERVOUS DISEASES OF CHILDREN. 

will be seen from this that the musculo-spiral nerve in the 
upper extremity, and its homologue, the peroneal nerve, in 
the lower extremity, are the nerves first attacked by the 
disease in a large number of the cases. This peculiar pre- 
disposition of these nerves to the disease cannot be ex- 
plained on the ground of anatomical position, and for the 
present is as inexplicable as are the facts of elective affin- 
ities in so many other diseases. 

In some cases the nerves supplying the trunk muscles, 
including the diaphragm, may be affected, but this is, after 
all, a very great rarity. A paralysis of the vocal cords 
due to multiple neuritis is quite as rare as is also an in- 
volvement of the facial nerve and of the hypoglossal. I 
have, however, seen a number of cases, particularly after 
diphtheria, in which the ocular muscles, and these only, 
were paralyzed from neuritis of the third and sixth nerves. 

The paralysis, although it may involve a number of groups of muscles, 
does not affect all equally ; those less affected may undergo contracture, such 
contractures occurring quite as often in the lower limbs as in the upper 
extremities. Deformities quite as serious and quite as persistent as those in 
poliomyelitis may therefore result from a chronic multiple neuritis. 

As regards the sensory symptoms it is important to 
note that every form of sensation is about equally impaired ; 
touch, pain, temperature, and muscular sense may suffer 
equally except in the earlier stages, in which a hyper- 
esthesia is a more common than hypassthesia. In a few 
instances the pain sensibility may be diminished while the 
sensation of touch remains, and even if the sensibility to 
pain is intact the conduction of pain impressions may be 
very much delayed. While pain and paralysis are the dis- 
tinguishing symptoms of a multiple neuritis it should be 
remembered that in not a few instances one or the other 
may be absent, and in some cases pain may never have been 
present to any marked degree, or may have lasted for so 
short a time as compared with the paralysis that the pa- 
tient who is examined at a later stage of the disease fails to 
remember that pain was ever present. The electrical re- 
actions may exhibit every form of change from a very 
slight diminution of faradic and galvanic response to a 



MULTIPLE NEURITIS. 245 

partial or complete reaction of degeneration. Atrophy is 
also a prominent and early symptom. The entire absence 
of bladder and rectum symptoms is of great importance in 
the diagnosis of multiple neuritis. 

Vasomotor changes in the paralyzed parts constitute a 
frequent symptom of multiple neuritis. (Edema and glos- 
siness of the skin, which are observed in the majority of the 
cases of peripheral nerve disease, are present in many cases 
of multiple neuritis. If the hand is involved we have not 
only the changes just mentioned but also a peculiar taper- 
ing of the fingers, which is quite characteristic of disease of 
the peripheral nerves. 

The course of the disease will vary very much according 
to the intensity of the cause. As a rule the symptoms in- 
crease during the first four to six weeks and then diminish. 
While the disease is increasing sensory symptoms are par- 
ticularly prominent. As soon as the acme of the disease 
has been reached these symptoms become less troublesome. 
The motor paralysis is recovered from much less quickly 
than are the disturbances of sensation. This is due not 
only to the actual loss of innervating power but to the 
atrophy of the muscles and to the contractures which have 
been formed. But even in severe cases a favorable change 
sets in sooner or later. The atrophy diminishes and the 
strength of the paralyzed limbs slowly increases until com- 
plete recovery is established. 

In some instances the onset of the disease is a very sudden one. The 
paralysis spreads rapidly and may resemble the course of Landry's paralysis ; 
but the fact that in multiple neuritis the trunk muscles generally escape and 
that the paralysis jumps from the lower extremities to the upper without in- 
volving the trunk, will help to differentiate it from an acute ascending palsy. 
While we have good reason to expect every case of multiple neuritis in a 
child to lead to recovery, death may result in consequence of complicating 
conditions, such as paralysis of respiratory muscles, or of the heart from 
disease of the vagus. Bronchitis and pneumonia are complications very 
much to be feared in children who have any form of multiple neuritis. Cir- 
rhosis of the liver and gross cerebral disturbances, due to alcoholism, which 
are common causes of death in cases of multiple neuritis in the adult, do not 
play a similar role in children. On the other hand, the involvement of the 
kidneys in cases of diphtheritic palsy is. a frequent cause of death. The 
psychoses described in connection with the multiple neuritis of adults are not 
met with in children. 



246 THE NERVOUS DISEASES OF CHILDREN. 

Multiple neuritis may result from a number of different 
causes: I. Toxic forms, including those due to metallic 
and non-metallic poisons. Among the former lead, arsenic, 
and alcohol are the most potent; and in rare instances 
mercury, carbonic oxide, and phosphorus may bring on a 
neuritis. In a very careful article in Keating's " Encyclo- 
paedia," Dr. Putnam, of Boston, has discussed the palsies of 
children due to lead and arsenic. He finds that poisoning 
by arsenic occurs most frequently through the mixtures 
used to destroy vermin, such as " Rough on Rats," " Paris 
Green," " German Fly-paper," and the like. Furthermore, 
arsenic is contained in many of the coverings used for 
books, toys, confectionery, labels, etc., as well as for arti- 
ficial flowers. The same author also believes that wall-paper 
and some special forms of carpet may be the source of poi- 
soning by arsenic. The arsenic is supposed to be commu- 
nicated to the child in the form of dust distributed in the 
currents of air, or in the form of some volatile compound 
which may be formed in a room which contains arsenious 
wall-paper. 

That some cases occur from the continued administration of arsenic in 
diseases like chorea there is very little doubt, and it is well to watch children 
carefully, while they are undergoing this form of treatment .* As for lead, it 
may enter the system from glazed cards, acid fruits and vegetables put up in 
tins, also from chocolate and other articles wrapped in tinfoil. Lead also 
enters largely into the composition of rubber, and has been found in the rub- 
ber nipples of nursing-bottles. Various kinds of cakes and candies are 
treated with lead, and prove a prolific source of poisoning in children ; but 
children as well as adults are exposed to lead-poisoning very much more fre- 
quently through drinking-water than in any other way. This is more com- 
mon in country districts, where lead pipes are connected with pumps, or 
where lead pipes are used to convey water from wells or springs at varying 
distances to the house. In the large cities this special source of lead-poison- 
ing is extremely rare. 

Alcoholic poisoning in children would naturally be con- 
sidered a rare condition, and so it fortunately is : but 
among the ignorant classes of every large population par- 
ents will be found who take special delight in the fact that 
their child can partake of everything, including beer or 

* I have seen one case of neuritis in the adult after the use of an arsenic paste over 
a large denuded surface. 



MULTIPLE NEURITIS. 247 

whiskey. A case of alcoholic neuritis in a child five years 
old has recently been reported from Strumpell's clinic. 
The child was raised in the saloon of its father, and not 
only drank considerable wine, but also two quarts of beer, 
every day. The father is quoted as saying that " the child 
was always thirsty, and of course we could not give it 
water." 

2. The toxasmic forms of multiple neuritis are far more 
important — at least in children. Among these we class, first 
of all, the septic forms of multiple neuritis which may occur 
in children as in adults after injury of any sort. The inva- 
sion of micro-organisms is unquestionably the direct cause 
of the neuritis. 

A similar invasion of specific organisms would account for the neuritis of 
leprosy and of beriberi, but these are so rare in this country, and particularly 
rare in children, that we need not make any further mention of them. 

Malarial neuritis is of great interest to us, for though it 
has been described chiefly by authors in Eastern countries, 
it is not unknown in these parts. I have seen several cases 
of this description in children who were brought to my 
clinic from the swampy parts of Long Island. 

Dr. Browning, of Brooklyn, has reported several such 
cases, which were supposed to be cases of poliomyelitis. 
These cases resemble poliomyelitis in their onset and devel- 
opment, and may be easily mistaken for it. In its general 
symptoms this form differs but very little from other cases 
of multiple neuritis except that it is associated much more 
frequently with the intermittent form of fever, that the dis- 
ease itself undergoes marked remissions, and that the en- 
largement of the spleen and examination of the blood are 
likely to give positive proof of the origin of the neuritis. 
Recovery is a little more rapid, too, in these cases than in 
those due to other causes. 

Among the toxasmic conditions which give rise to mul- 
tiple neuritis we must include those blood states which fol- 
low upon the various acute infectious diseases (diphtheria. 
small-pox, typhoid fever, tuberculosis, and also syphilis). As 
the nerve affection is developed a long time after the initial 
infection it is probable, in the light of recent bacteriological 



248 THE NERVOUS DISEASES OF CHILDREN. 

researches, that some chemical product of the micro-organ- 
isms, and not the bacteria themselves, are the direct cause 
of the neuritis. With the exception of diptheritic multiple 
neuritis (p. 253) all the forms just mentioned are compara- 
tively rare in children. 

In obedience to the ordinary belief, we must concede the 
existence of the" rheumatic forms of multiple neuritis ; but I 
am free to confess that I have seen only a very few cases of 
multiple neuritis in children which could with some degree 
of probability be referred to rheumatic causes. In cases of 
articular rheumatism in children the nerves are sometimes 
involved, as is proved by pains radiating down the limb 
through special nerve-trunks. Refrigeration neuritis occurs 
in children. In one instance this was due to ice-cold baths 
which a mother gave to her child daily all the year around. 
Now and then cases come under one's notice of severe forms 
of polyneuritis in which the true cause cannot be ascer- 
tained. 

Pathological Anatomy. — The chief lesions are re- 
stricted to the peripheral nerves. It is natural to infer that 
the changes are very much the same as those to be found 
in cases of ordinary simple neuritis (see page 217). In mul- 
tiple neuritis the changes are chiefly of a parenchymatous 
order, or interstitial in character ; the perineurium is not 
often involved. In some cases, however, the nerve is swol- 
len, the nerve-sheath is hyperaemic, of a deep purplish 
color, and, according to Eichhorst, may be covered with 
minute hemorrhages. There may be some slight changes 
also in the consistence of the nerve. All these conditions, 
and the appearances of degeneration, will vary according 
to the stage of the disease at the time of examination. In 
the earlier stages we often find the nuclei of the fibres en- 
larged, a proliferation of the connective tissue between 
the nerve-elements, and numerous round or spindle-shaped 
cells with myeline granules near the sheath of the nerve. 
If the specimen is examined at a later period of the disease 
the acute condition will have passed and the nerve presents 
the simple appearance of subacute or chronic degeneration 
of nerve-fibres. (Fig. 63.) 

In some cases the muscular tissue is slightly involved, 



MULTIPLE NEURITIS. 



249 






the fibres are smaller and paler, the transverse striation is 
less distinct than under normal conditions, while the nuclei 
of the fibres and of the interstitial tissues may be increased. 
The changes in the muscle may be both parenchymatous 
and interstitial. 

In considering the pathology of multiple neuritis, the 
most striking fact is the peculiar action of toxic agents upon 
the peripheral nerves, without affecting the spinal centres. 
The farther away the nerve-fibre is from the mother-cell 
the less able it is to resist morbid agencies ; for this reason 
the more distal parts of the peripheral nerves are affected 
at an earlier period in multiple neuritis than are the parts 
of the nerve nearer 
the spinal centres. 

This argument is 
strengthened by the re- 
cent conceptions of the 
close relationship between 
the ganglion-cell and the 
peripheral nerve -fibre as 
parts of a neuron. More- 
over, recent researches 
have shown that the same 
poison which often affects FlG 63 ._ Chronic interstitial Neuritis with Atrophy of 
the peripheral nerve-fibre 
may occasionally involve 
the ganglion-cell. Stieglitz 
has proved this to be the 
case in lead-poisoning. He 
found a distinct poliomy- 
elitis in animals compelled 
to inhale lead salts ; yet we cannot deny, in view of various post-mortem 
findings, that in man lead is prone to cause disease of the peripheral nerves. 
Oppenheim has described slight changes in the spinal cord in cases of alco- 
holic neuritis, and I have little doubt that similar changes will be made out 
in cases of arsenical neuritis. 

Diagnosis. — With our present knowledge of the charac- 
teristic symptoms of multiple neuritis there should be no 
difficulty in differentiating between the various forms oi 
this disease and other diseases with somewhat similar 
symptoms. The chief characteristic of these peripheral 
nerve diseases is the association of sensory with motor 




some Nerve-fibres. (Ziegler. ) a, cross-sections of 
normal nerve-fibres ; b, cross-section of thin, but nor- 
mal, nerve-fibres ; c, endoneurium ; d, cellular in- 
filtration and proliferation of connective-tissue strands 
and blood-vessels ; f, thickened endoneurium with 
small empty nerve-spaces and some normal thin nerve- 
fibres ; g, longitudinal section of a blood-vessel. 



250 THE NERVOUS DISEASES OF CHILDREN. 

symptoms, the distribution of both pointing to an in- 
volvement of the same nerve-areas. Persistent sensitive- 
ness of the nerve-tracts and subjective pain in the course of 
the peripheral nerves help to corroborate the diagnosis. It 
is easy to confuse multiple neuritis with poliomyelitis, and 
until recent years this mistake was made by the ablest diag- 
nosticians ; but in poliomyelitis the initial symptoms are 
of a rather more violent character, and the entire central 
nervous system often shows the influences of the disease 
very much more than it does in multiple neuritis ; the 
paralysis is more symmetrical in neuritis than in poliomye- 
litis. 

In poliomyelitis there is no pain along the nerve-tracts and these nerve- 
tracts are not sensitive, but vague pains are very often present in the first 
week or two of poliomyelitis. [ have the record of a child, two years old, 
whom I saw two days after the onset of the paralysis, in whom the entire 
limb was so painful that the child would scream violently if the limb was 
suddenly touched, and this was not due to the fear of examination. The pain 
was not distributed along any nerve-paths, and I did not hesitate to make 
the diagnosis of poliomyelitis, which proved to be the correct one by the 
subsequent course of the disease. The electrical reactions are very much 
the same in both disorders. 

The difficulties of this problem are increased by the fact that, as Gowers 
has reported, cases occur in which a typical anterior poliomyelitis is compli- 
cated by a peripheral neuritis. In spite of all efforts at differentiation cases 
will occur every now and then in which it will be impossible to make an ac- 
curate diagnosis at the start, and the physician must watch the course of the 
disease before coming to a definite conclusion. 

From pachymeningitis, which is rare in children, the disease may be dif- 
ferentiated more easily, since in the former the legs are rarely affected, the 
anaesthesia is apt to spread to the upper limbs and to the trunk of the body, 
and there is no distinct sensitiveness to pressure on the part of the larger 
nerve-trunks, except late in the disease, and the atrophy is restricted to 
muscles innervated from the diseased spinal segment. 

Landry's paralysis is to be recognized by the rapidly ascending palsy pro- 
ceeding from segment to segment, involving the abdominal and thoracic 
muscles, and not skipping from the lower to the upper extremities, as is the 
case in poliomyelitis. The differentiation between the ataxic forms of 
multiple neuritis or the so-called pseudo-tabes from true tabes need hardly 
be considered in the case of children, as in them true tabes is a disease of 
exceeding rarity. But it may be well to bear in mind that the symptoms of 
hereditary ataxia (Friedreich's disease), including the loss of knee - jerk, 
ataxic gait, weakness, and awkwardness in walking, may bear a superficial 
resemblance to polyneuritis ; but the gradual development of the symptoms in 



MULTIPLE NEURITIS. 25 I 

this hereditary disease, the occurrence in various members of the same family, 
the peculiar appearance of the face, the disturbance of speech, the mental 
peculiarities, will render the diagnosis positive enough. 

As children are subject to hysterical palsies the question of differential 
diagnosis may occasionally arise, but hysterical palsy is not characterized by 
the loss of reflexes ; nor is the ataxia, if present, like that of polyneuritis ; and, 
moreover, the anaesthesia accompanying the palsy in hysterical cases is of 
the regional order, and not in keeping with the anatomical distribution of the 
nerves. If there is any reason to suspect myelitis, the presence or absence 
of bladder and rectal symptoms, the development or non-development of bed- 
sores will weigh heavily for or against the diagnosis of cord disease. 

Trichinosis must be suspected in cases of great painfulness of the ex- 
tremities, but the other attendant symptoms (gastric disturbances) and the 
excessive swelling of the muscles will help to clear up the diagnosis. 

The recognition of the cause of multiple neuritis is an important point in 
the diagnosis of the disease. In this country the most frequent cause will 
undoubtedly be found to be a preceding acute infection, either by poisoning 
or by the toxic products of some acute infectious disease, such as diphtheria, 
variola, and typhoid fever. Next in frequency I would place the probability 
of a malarial origin, and last, the possibility of tubercular, syphilitic, or alco- 
holic infection. 

Treatment. — First of all we must determine the pri- 
mary cause of the disease, and, if possible, the deleterious 
agent must be removed or its influence arrested. If the child 
lives in a malarial district it should be taken to a place 
where there is no danger from further malarial infection. 
If there is any suspicion of alcoholic, or of lead, or of arseni- 
cal poisoning, the sources from which such poisons have 
emanated must be absolutely cut off. If some acute infec- 
tious disease has preceded, we can, of course, do nothing to 
prevent the natural course of the neuritis, but b} 7 improving 
the child's general health will enable it to conquer the dis- 
ease much more rapidly. For the relief of pain, which is 
by far the most annoving symptom at the beginning of the 
disease, the application of heat is the best remedy we have. 
If heat is applied at later stages of the disease, when con- 
siderable anaesthesia may be present, it is of the greatest 
importance to watch the condition of the skin, or else seri- 
ous burns and ulcerations may result. It is on this account. 
also, that the use of the actual cautery and of other forms 
of counter-irritation is not to be recommended. Prolonged 
warm baths will often help to alleviate the suffering, and 



252 THE NERVOUS DISEASES OF CHILDREN. 

will contribute largely to the comfort of the patient. If 
the pain is so intense that it cannot be relieved by these 
simple measures it will be well to give small doses of mor- 
phia, codeia, or rectal injections of chloral hydrate. 

There is no drug which has any specific action upon 
multiple neuritis. I have given the salicylates invariably, 
but am not convinced that they have done much good. 
Mercurials have been praised by some, particularly in cases 
in which the nerve-sheath is involved rather than in the 
parenchymatous form, yet I have not been able to persuade 
myself of the benefits to be derived from mercurials, even 
in many cases of multiple neuritis in the adult ; but if there 
is any good reason for the employment of mercury let it be 
given in the form of inunctions of unguentum hydrargyri 
or of a ten or twelve per cent, oleate of mercury. 

The administration of mercury, either by the mouth or 
in the form of hypodermic injections, is to be avoided in chil- 
dren, if possible. Iodide of potash or iodide of sodium has 
little or no influence upon the disease ; arsenic, which has 
gained such popularity in every form of chronic nerve dis- 
ease, is scarcely a safe remedy to use, for in small doses it 
will have no effect, and if given in large doses there is a de- 
cided danger of increasing the neuritis. We are compelled, 
therefore, in all cases of multiple neuritis in children, par- 
ticularly in those occurring after diphtheria and other acute 
infectious diseases, to resort to general tonic remedies. 
Among these none is better than cod-liver oil, and next in 
order, though far inferior to it, are iron, quinine, and 
strychnia in very small doses. In all cases of malarial 
neuritis, quinine should be given in the same doses that 
would be administered to combat other symptoms of mala- 
rial poisoning. A word of caution should be added with 
reference to the use of anodynes. In children the careless 
exhibition of morphia may result in the formation of the 
morphia habit, and morphia itself, if given in any consid- 
erable quantities, may help to intensify rather than to 
diminish the symptoms. 

If insomnia is persistent in consequence of the pain in 
the initial stages, one of the newer hypnotics, such as chlor- 
alamid, trional, or sulphonal, in doses varying between five 






MULTIPLE NEURITIS. 2$$ 

and fifteen grains, according to the age of the child, should 
be given a trial ; if they do not produce sleep, codeia in 
doses of one-sixth to one-half of a grain may be adminis- 
tered. 

After the first two or three weeks of the disease the 
paralytic symptoms become rather distinct. In all those 
cases in which there is considerable paralysis with atrophy 
the use of the galvanic current, both as a sedative and as a 
means of exercising the paralyzed parts should be em- 
ployed. The faradic current will be of little service if it 
fails to produce contractions, and as it has a distinct irritat- 
ing effect upon the sensory filaments in the skin it should 
not be applied. When all pain has disappeared and the 
progress of the disease has been stayed, light massage can 
be employed to advantage ; it is specially to be recom- 
mended at the time when contractures are about to be de- 
veloped, or when the atrophy of the muscles is on the in- 
crease. If permanent contractures result from multiple 
neuritis surgical interference may be necessary, but as 
the disease generally leads to spontaneous recovery, every 
possible means of bringing this about should be employed 
before the child is given over to the surgeon ; yet I can 
see no harm in having a simple tenotomy performed if that 
will enable the child to bring its feet to the ground, or to 
walk before complete recovery from all the symptoms has 
set in. In some of the cases some simple orthopedic ap- 
paratus will help the child to learn to walk, and will enable 
it to use muscles which cannot be depended upon to sup- 
port the body ; but there is also a danger in allowing chil- 
dren to wear an apparatus too long a time, as they are very 
prone to depend entirely upon such artificial support, and, 
as a rule, lack the energy to exercise weakened muscles. 

DIPHTHERITIC PARALYSIS. 

Paralysis after diphtheria occurs in a very large percent- 
age of all the cases, and is relatively more frequent after 
diphtheria of the adult than after the same disease in earlier 
life. In earliest infancy, say up to the age of two years, 
diphtheritic palsy is extremely rare. It bears no absolute 



254 THE NERVOUS DISEASES OE CHILDREN. 

relation to the severity of the diphtheritic infection. 1 have 
often seen typical diphtheritic palsy set in after an infec- 
tion so slight that it was considered to be nothing but a 
mild sore throat ; but, on the other hand, it also occurs in 
connection with severe diphtheritic infection, and is fre- 
quently associated with other complicating diseases, such 
as nephritis and endocarditis, following upon the original 
disease. The previous health of the child has no distinct 
bearing upon the development of the palsy, strong and 
weak children being affected with equal frequency. I have 
seen the paralysis developed within the first week, while the 
throat was still covered with diphtheritic membrane, but 
the majority of cases do not occur until two, three, or four 
weeks after the termination of the disease. 

Diphtheritic palsy differs from other forms of multiple 
neuritis in the order in which the various parts become 
paralyzed. The palate is generally the first, often the only, 
part affected. Nasal speech and regurgitation of liquids 
through the nose are evidences of paralysis of this part, and 
the loss of function can be determined by an inspection of 
the mouth and throat. Loss of accommodation is next most 
frequent. If the paralysis extends further, the upper and 
lower extremities are apt to be involved. At first a mere 
Aveakness is noticeable, associated with tingling and numb- 
ness of the parts; later on this weakness increases, and 
develops into a full-fledged paralysis, and the disturbance 
of sensation may become more marked. The ocular mus- 
cles show a peculiar predisposition to diphtheritic infec- 
tion ; the rectus externus, supplied by the sixth nerve, is 
frequently, and often singly, affected, and the oculo-motor 
nerves are also involved at times, but a complete ocular 
motor palsy in diphtheria is rare. Ptosis and paralysis of 
one or more muscles supplied by the third nerve have fre- 
quently been noticed. The optic nerve fortunately escapes, 
so that blindness is not a common result of diphtheria, un- 
less by some untoward accident the diphtheritic poison 
should cause ulceration or purulent disease of the eye, with 
subsequent loss of vision. The ciliary muscle is often par- 
alyzed ; the reaction of the pupils to light is sluggish, but 
the contraction during accommodation may be preserved, 



MULTIPLE NEURITIS. 2$$ 

although the act of accommodation itself is carried out im- 
perfectly. 

Paralysis of the pharynx may occur, but is not frequent ; 
the larynx, however, often comes in for a share of the pa- 
ralysis ; the epiglottis then fails to perform its function, and 
food may reach the larynx and cause severe fits of cough- 
ing. Hoarseness and imperfect phonation point to an in- 
volvement of the muscles of the larynx and of the vocal 
cords. This special order of paralysis is generally sym- 
metrical. 

The reflexes are diminished or lost, even when no dis- 
tinct paralysis of the adjacent parts exists or existed (Bern- 
hardt). I have had opportunity to examine four children 
whose knee-jerks were absent after diphtheria, but who 
had never presented any paralytic symptoms. The same 
condition is found occasionally after scarlatina and typhoid 
fever. 

In the limbs the change in the motor and sensory functions is very like 
that of other cases of multiple neuritis, and does not, thererefore, require 
any special mention. It is questionable whether cardiac failure coming on 
after diphtheria is due to a neuritis of the pneumogastric, or whether, as 
Leyden would have it, it is due to a degeneration of the heart muscle itself. 
The irregularities of respiration associated with the heart symptoms are so 
characteristic of vagus affections that it seems much more probable to attrib- 
ute the loss of function to disease of the nerve rather than of the muscular 
tissue. 

Some diphtheritic palsies occur which do not in any way resemble mul- 
tiple neuritis ; a hemiplegia may follow upon diphtheria, but this may be the 
result of a vascular lesion so common in connection with other infectious dis- 
eases of childhood. Cases of this character have been described by Mendel 
and Bonath. 

Paralysis of the masseters has been reported by Grant and quoted by 
Gowers. As the paralysis was permanent fourteen months after the disease 
Gowers thinks the affection due to an acute nuclear inflammation. A degen- 
eration of various cranial nerves has been observed to come on after a con- 
siderable lapse of time, following upon diphtheria, but it has seemed a little 
doubtful whether such paralysis could be traced directly to a diphtheritic poi- 
son. 

The general course of the disease leads to recovery. It 
may last from six to eight weeks ; the cases in which single 
nerves only are affected recover more rapidly than those 
with multiple nerve-lesions. The ocular nerves recover 



256 THE NERVOUS DISEASES OF CHILDREN. 

very much more quickly than the nerves of the lower ex- 
tremities do. If there is paralysis of the legs, it takes, as a 
rule, from four to six months before the symptoms disap- 
pear. The reflexes are late in returning, and in most cases 
cannot be elicited until some weeks after all other symp- 
toms have passed away! 

If death ensues during diphtheritic palsy, it is either due to some compli- 
cating disease, such as nephritis or pneumonia, or to paralysis of the heart 
or respiratory muscles. Mere exhaustion from inability to take food in cases 
of paralysis of the pharynx is an occasional but rare cause of death. 

Pathological Anatomy. — The morbid changes are 
essentially the same as those found in other forms of multi- 
ple neuritis ; the inflammatory and degenerative changes in 
the nerves are in these cases probably the result of micro- 
bic poisoning, or the effect of chemical poisons formed by 
the diphtheritic micro-organisms ; but bacteriological re- 
searches have not yet yielded a very satisfactory explana- 
tion of all the changes that take place in the peripheral 
nerves. The rod-shaped bacteria, the bacilli of Klebs and 
Loeffler, have been found in the blood-vessels of the nerve- 
centres, and other micrococci have been described by Oer- 
tel as occurring in these vessels ; but further study Avill be 
needed to explain the origin of the inflammatory process in 
the nerves. The degeneration is found in the nerve supply- 
ing the paralyzed part ; often the entire nerve is affected, at 
times some of the filaments only. In others, the degenera- 
tion extends to the anterior or even the posterior roots. 
The myelin and nerve-filaments undergo segmentation, 
the nuclei of the sheath are increased, and granular corpus- 
cles mark the decay of nerve-tissue. The axis-cylinder 
remains intact until the destruction of the medullary sheath 
has advanced considerably, and both these parts may then 
be entirely destroyed. The interstitial tissue between the 
nerve-fibres is but little affected, thus marking the condi- 
tion as a true parenchymatous degeneration. The nerves 
supplying the palate are most frequently and most severely 
affected, but the same changes may occur in the peripheral 
nerves of the extremities in all the ocular nerves, and even 
the phrenic nerve may be similarly affected. 









MULTIPLE NEURITIS. 2$J 

In diphtheritic paralysis the muscular tissue is more distinctly changed 
than in other forms of paralysis due to multiple neuritis. In cases of long- 
standing the muscular fibre of the palate, for instance, is found to have under- 
gone granular and fatty degeneration. In some there are signs of parenchy- 
matous as well as of interstitial inflammation, and every possible grade may 
be found between a simple inflammatory condition and complete degenera- 
tion of the muscular fibres. In cases in which the disease leads to implica- 
tion of the heart muscle, the pallor of the heart after death is the external 
evidence of complete degeneration of its muscular fibres. 

Much has been made of changes in the spinal cord in cases of diphthe- 
ritic paralysis, but these are restricted entirely to changes of the ganglion cells 
of the anterior horns, which have been found swollen, homogeneous in 
character, and with processes somewhat altered or entirely shrunken ; but it 
is doubtful whether these changes are primary or secondary, though there is 
no good reason why a poison which acts so vigorously upon the peripheral 
nerves should not invade the various tracts of the spinal cord. 

The frequent disease of the palate is probably due to the proximity of 
the nerves supplying the palate, to the diphtheritic membranes, and the eas- 
ier invasion into these nerves of the micro-organisms lodged in the tonsils, 
or in the back of the throat. And yet if we remember that a considerable 
period of time may elapse between the deposit of the diphtheritic membranes 
in the throat and the appearance of the palsy, and that the palsy may appear 
after very mild throat affections, it is not altogether easy to explain why these 
nerves should be so frequently the seat of the disturbance, unless we suppose 
that from their proximity to the original diphtheritic lesion the nerve-tissue 
has become altered and thus rendered more susceptible to the diphtheritic 
microbes, or to the microbic toxins. The poison attacks other nerves in 
the course of time ; the peroneus, the musculo-spiral, and the median are the 
most susceptible to it. 



The diagnosis of this palsy often depends upon the 
history of preceding diphtheria, or of some throat affection, 
however slight it may have been. The beginning of the 
palsy in the palate, and gradually spreading to the lower 
and upper extremities, will at once suggest the probability 
of diphtheritic paralysis. It is only in cases in which the 
original disease has been overlooked or forgotten that an 
examination of the patient some weeks after the onset of 
the trouble may, through the atrophic paralysis and the ab- 
sence of knee-jerk, suggest spinal infantile palsy ; but a 
closer examination of the patient, and the comparatively 
rapid development of the palsy, with its tendency to recov- 
ery, will place the diagnosis beyond doubt. Diphtheritic 
palsy, like other forms of paralysis in children, may con- 
17 



258 THE NERVOUS DISEASES OF CHILDREN. 

tinue for an inordinate length of time, and may be super- 
seded by an hysterical palsy. Thus it happens not infre- 
quently, as in the daughter of a clergyman whom I examined 
a few years ago, that a chronic paralysis is the outcome of 
a diphtheritic palsy which had set in a few weeks after a 
severe throat affection. 

The prognosis in diphtheritic paralysis is tolerably 
good, if we except the possibility of cardiac and renal 
complications. The paralysis of the palate, however dis- 
agreeable it may be to the child, rarely leads to serious 
complications, but if the muscles of the pharynx are in- 
volved there is danger of food entering the respiratory 
tract, and of a subsequent pneumonia. 

Other things being equal the earlier the paralysis appears after the initial 
disease, and the more quickly it reaches an extreme development, the more 
serious the case and the more imminent the danger to life. It is difficult to 
forecast the exact length of time which will elapse before complete recovery 
sets in, but it is safe on the whole to be guided by the same rules as in the 
case of any other peripheral nerve palsy. If the electrical examination shows 
an entire loss of response to the faradic current with an altered response to 
the galvanic current, many weeks will in all probability elapse before recov- 
ery sets in. If there are no marked changes in the electrical conditions of 
the nerves and muscles the degeneration or the inflammation of the nerve is 
very slight and the paralysis will be recovered from more quickly. 

Treatment. — The majority of cases of diphtheritic 
palsy will get well without any treatment, but it is of the 
utmost importance at all times to maintain the strength of 
the patient at par, and to be prepared for any sudden com- 
plications. If the palate is paralyzed and there is regur- 
gitation of food through the nose it will be better to feed the 
child by solids than by liquids ; if in consequence of paraly- 
sis of the pharynx and of the upper air-passages there is 
danger of food entering the respiratory tract it may be best 
to feed the child for a time per rectum, or else to use the 
feeding-tube through the nose or the mouth. In the case 
of children weakened by the diphtheritic process there is 
danger of death from exhaustion, and no time should be 
lost in employing this method of feeding rather than to 
starve the child while hoping for a spontaneous recovery. 
On the other hand, even while the rectal feeding is kept up 



MULTIPLE NEURITIS. 259 

efforts should be made every now and then to have the 
child take its food in the natural way. If in any case of 
diphtheritic palsy there is the slightest irregularity or weak- 
ness of the pulse, cardiac stimulants should be given, above 
all digitalis and small doses of strychnia. If respiratory 
weakness threatens, the prompt use of the faradic current, 
as advised by Duchenne, is quite in order. Direct excita- 
tion of the phrenic nerves by a slowly interrupted faradic 
current will have the best results. The electrical currents 
should be employed, according to the principles laid down 
in other chapters, for the treatment of paralyzed parts. 

The usual tonics — iron, quinine, arsenic, digitalis, and 
strychnia — should be given, but no one would venture to 
assert that any one of these has any special curative action 
except in the way of keeping up the general condition. 
Indeed there is no better method of treating diphtheritic 
palsy than by carefully administering food, and making 
sure that everything taken into the system will help to 
improve the nutrition of the child.* 

LEAD PARALYSIS. 

A few pages are to be devoted to this special form of paralysis, in order to 
emphasize the fact, which was proved conclusively by Dr. Putnam, that chil- 
dren are no less susceptible than adults to the effects of lead-poisoning. Dr. 
Putnam has not only shown that entire families have been poisoned by lead, 
but in a series of examinations, made for the purpose of determining the 
presence of lead in the urine of persons who were supposed to be healthy, he 
found considerable quantities in the urine of father, mother, and two of the 
children of one family, all of them being in fair health ; a third child, how- 
ever, for some unknown reason, did not exhibit any traces of the poison. 
The water drank by this family ran for some distance through lead pipes 
and was found to contain a large quantity of lead. If present in small quan- 
tities, lead is practically harmless. Knowing the varied susceptibility of 
different persons to lead, and having proved its presence in the urine of per- 
sons who exhibited no symptoms of lead-poisoning, Dr. Putnam is right in 
urging that the possibility of lead-poisoning should be taken into considera- 
tion in all obscure cases. 

As for the symptoms of lead-poisoning in children, they differ but little 
from those presented by adults, and are in entire keeping with the symptoms 

* Since the above was written, Behring's important discoveries have been made 
known. Whether diphtheritic palsy can be affected by injections of the serum remains 
to be seen ; but it is to be hoped that paralytic symptoms will become less frequent 
after injections of the serum for the treatment of the earliest stages oi diphtheria. 



260 THE NERVOUS DISEASES OF CHILDREN. 

of multiple neuritis due to other causes. The paralysis is most frequently 
developed in the extensor groups of the forearm, resulting in characteristic 
drop-wrist (Fig. 62 ). The muscles affected are supplied chiefly by the mus- 
culo-spiral nerve ; the supinator longus, and the extensor of the thumb usu- 
ally escaping. This escape of the supinator longus is frequently of service in 
helping to distinguish the disease from a simple traumatic musculo-spiraL 
neuritis, for in the latter case all the muscles supplied by this nerve are about 
equally affected, whereas lead, like other toxic agents, such as alcohol and 
arsenic, shows a selective preference for groups of muscles that are function- 
ally, not structurally, related. In adults it is rare to find other muscles seri- 
ously affected, except those of the upper extremities, although if careful 
examination is made of such patients, a slight weakness in the lower extrem- 
ities and a diminution of the reflexes will be found to be present. I have 
myself seen two cases of lead palsy in the adult in which the entire affection 
was restricted to the lower extremities. In one case the adductor muscles of 
the thighs were the only ones affected. In children the involvement of the 
lower extremities is not exceptional, and when it does occur, is just as apt as 
not to occur earliest in the course of the disease, the upper extremities being 
affected after the lower extremities have been paretic or paralyzed. 

While the symptoms are practically identical with those of the adult, the 
diagnosis of lead paralysis will be all the more readily impressed upon the 
physician by the occurrence of other symptoms. Thus the child is apt to ex- 
hibit a general cachectic appearance ; also a peculiar ashen hue of the skin , 
severe headaches, and marked disturbances of digestion. Soltmann expressed 
the opinion some years ago that lead colic was rare in children. It is more 
correct to assert that it is difficult to distinguish lead colic from other forms 
of colic so frequent in children. Disturbance of digestion due to constipation 
is the rule, but constipation may alternate with diarrhoea and with severe 
attacks of colic ; the colic being distributed over the entire abdomen and not 
in the vicinity of the umbilicus, as is generally the case in the adult. Vomit- 
ing frequently occurs, and the vomitus may be yellowish in color. The ab- 
domen may be retracted, but in many of the cases one or all of these symp- 
toms may be absent. 

If the lead poison attacks the brain, the tendency to convulsions will be 
very pronounced, and convulsions may possibly be the cause of death. In the 
cases reported by Karsch and Stewart (quoted by Putnam), the children died 
in convulsions, one of them with a mild form of delirium. Gowers supposes 
that epileptic attacks may occasionally originate in lead-poisoning, but Put- 
nam, whose experience in this matter is greater than that of any other author, 
is inclined to think that such a sequence of events has not been firmly proven. 

The lead line, which is of such assistance to us in diagnosticating lead- 
poisoning in the adult, is not so frequently observed in children, although in 
one epidemic forty-two cases of the sort were reported. The better condition 
of the teeth in children is unquestionably the explanation of this. 

The pathology and pathological anatomy of lead-poisoning are still sub- 
jects for discussion. That lead acts most powerfully on the peripheral nerves 
cannot be doubted, and that in the majority of cases lead paralysis is the ex- 



MULTIPLE NEURITIS. 26 1 

pression of a neuritis it is also safe to assume. Gombault insists that lead 
causes a periaxillary neuritis, the sheath of the nerve being more affected 
than the axis-cylinder and relatively healthy portions of nerve intervening 
between diseased parts. These changes were found in guinea-pigs that 
were not paralyzed, and Putnam suggests very correctly that such changes in 
the myelin without paralysis may account for the fact that in man the elec- 
trical reactions are very often altered before any actual paralysis appears. 
Harnack and others have suggested that lead has a direct action upon the 
muscles rather than upon the nerves, but the experimental researches of 
Stieglitz, published a few years ago, have brought the entire discussion to a 
temporary and satisfactory close by showing that lead produces changes in 
the spinal cord as well as in the peripheral nerves. 

Prognosis. — The prognosis of lead-poisoning is, as a rule, entirely favor- 
able. There is more danger from the general nutritional disturbances and 
from the effect of the poison upon the brain, than from the lesion of the 
peripheral nerves and of the spinal cord, which may have given rise to the 
paralysis. If the cachectic condition is recovered from it is more than likely 
that the patient will regain considerable power in the legs in the course of a 
month or two, but the actual time that elapses before complete recovery sets 
in may vary as much in lead palsy as it does in other forms of multiple 
neuritis, and it need not be a matter of surprise if a child poisoned by lead 
shows the effects of such poisoning for six months, or even a year ; but under 
all conditions it is a source of satisfaction to be able to predict complete re- 
covery eventually. 

Treatment. — If the patient is in the acute stage of lead-poisoning the 
same measures should be employed which are advised in other cases of acute 
metallic intoxication. If the patient is in a subacute or chronic stage, an at- 
tempt should be made to eliminate the poison through the kidneys and skin. 
The iodides have become the classical remedy. It is better to clear the sys- 
tem of the lead gradually than to attempt to eliminate the poison in a very 
few days. Doses of from five to ten grains of the iodides three times a day 
will be quite sufficient for all purposes, and these should be administered in 
some alkaline water which will stimulate the kidneys to greater activity. 
Warm baths should be given every day, or every other day, in order to help 
on the same cause. 

After everything has been done to eliminate the lead, the attention should 
be directed entirely to the treatment of the paralyzed parts, and in this re- 
spect the use of active massage and electricity, in the manner so often re- 
ferred to, is advisable. The general condition of the patient will also call 
for the active exhibition of blood tonics, such as iron, arsenic, and cod-liver 
oil. 

BIBLIOGRAPHY. 

Barrs : American Journal of the Medical Sciences, February. 1SS9. 

Bernhardt: Zeitschr. f. kl. Med., Bd. XL, p. 365. 

Blocq et Marinesco : Nouvelle Iconographie de la Salpetriere. 1S90. p. 137. 



262 THE NERVOUS DISEASES OF CHILDREN. 

Bonath : Neurolog. Centralblatt, 1893. 

Bramwell : American Journal of the Medical Sciences, June, 1888. 

Browning, William : American Journal of the Medical Sciences, December, 

1 891. Brooklyn Medical Journal, January, 1893. 
Brissaud : Arch. d. Neurologie, 1891, p. 161. 
Buzzard: Monograph, 1885. 
Eichhorst : Virchow's Arch., Bd. CXXVIII. 
Eisenlohr : Deutsches Arch, f. klinische Med., 1880. 
Gombault : Arch, de Physiologie Norm, et Path., 1873, p. 593. 
Gowers : Multiple Neuritis and Poliomyelitis. Reprint. 
Grasset et Rauzier : Vol. ii., p. 87. 
Hirt : Neurol. Centralbl., 1884, p. 21. 
Jacoby : New York Medical Journal, August, 1889. 
Joffroy: Archives de Physiologie Norm, et Path., vol. xi., p. 177. 
Jolly : Bed. Med. Ges., December 7, 1892. 
Kast : Arch. f. Psychiatric Bd. XI. 
Klumpke : Des Polyneuritis, These de Paris, 1889. 
Leyden : Zeitschrift f. kl. Medicin, Bd. I. 
Lloyd: Medical News, 1892. 
Meyer : Virchow's Arch., Bd. XCV. 
Mendel: Neurolog. Centralbl., 1885. 
Oppenheim: Zeitschr. f. kl. Med., 1885, Bd. XL, p. 232. 
Pitres et Vaillard : Rev.de Med., 1885,1886. Also, Arch, de Neurolog., 

1886. 
Putnam: In Keating's Cyclopaedia (Lead Palsy). 
Rosenheim : Arch. f. Psych., Bd. XVIII., 3. 

Starr: Middleton Goldsmith Lecture, New York Medical Record, 1887. 
Stieglitz : On Experimental Lead Palsy, Arch. f. Psych., Bd. XXIV. 
Striimpell : Arch. f. Psych., etc., 1883, Bd. XIV., 2. Berl. kl. Wochen- 

schrift, August, 1885. Neurolog. Centralbl., 1884. Muenchner Med. 

W., 1886, No. 34. Zeitschr. f. Nervenheilkunde, 1893. 
Vierordt : Degeneration d. Goll'schen Straenge, Arch. f. Psych., Bd. XVIII. 






DISEASES OF THE SPINAL CORD. 



CHAPTER XIV. 

ANATOMY, PHYSIOLOGY, AND PATHOLOGY OF THE SPINAL 

CORD. 

In this chapter it is proposed to give those facts merely 
which have an important bearing upon clinical diagnosis. 
Many details of the normal and morbid anatomy of the cord 
will, therefore, be omitted, however great the scientific in- 
terest attaching to tfiem may be. 

Structure of the Cord. — The spinal cord cannot be 
differentiated from the brain by any sharp line of demarca- 
tion. As a matter of convenience the former is said to be- 
gin below the decussation of the pyramids in the medulla ; 
but the medulla and pons are practically the extension of 
the spinal-cord structures, rather than integral parts of the 
brain proper. In-structure, in function, and in their relation 
to disease, the pons and medulla incline very much more 
toward the spinal cord than toward the brain. For all 
practical purposes, however, we may adhere to the accus- 
tomed plan of division, and may hold that the spinal cord 
begins back of the metencephalon, that is, below the part 
which gives rise to the medulla, and comprises all of the 
neural axis from this point to the filum terminale. 

The spinal cord of the child is not sufficiently distinct from that of the 
adult to call for any special discussion. The points which we wish to bring- 
out would, therefore, apply with equal force to the spinal cord of adult man. 
the difference in size and thickness remaining proportionate to similar differ- 
ences in the bony parts surrounding the cord. 

The only points to be noted are the following : In the foetus the cord at 
first occupies the entire length of the vertebral canal, but from the third 
month on, the roots of the lumbar and sacral nerves grow much more rap- 



264 



THE NERVOUS DISEASES OF CHILDREN. 



ci 



idly than the cord itself, so that at birth the lowest part of the cord is oppo- 
site to the third lumbar vertebra. After birth, the cervical and dorsal portions 
of the cord grow more rapidly than the other 
parts, so that in the infant the roots of the lower 
! c dorsal nerves come off relatively higher than at a 

• 2 later age (Schaefer and Fitzner). 

• 3 The spinal cord is not as long as the spinal 
4 column ; its lowest portion is generally taken to 

- 5 be situated opposite to the second lumbar verte- 
6 bra. For that reason a given spinal-cord seg- 
■ 7 ment is a little higher than the vertebral body 

• s through which the roots of any given segment 
•I D pass. Still more confusion is added thereto by 
•a the fact that the vertebral spines, which alone can 
. 3 be felt through the skin, are not always on the 

4 same level with the vertebras. Gowers has made 
a careful study of these relations, and the exact 
correspondence of the various parts is well dem- 
onstrated in the annexed figure (64). This rela- 
1 tion between the spinal vertebras and the seg- 
- 8 ments underneath has become a matter of much 
practical importance since operable diseases of 
the spinal cord have compelled the physician 
* 10 and the surgeon to localize disease of the spinal 
-" cord as accurately as it has long been their cus- 
. I2 torn to do with regard to many diseases of the 
brain. 



<a 






12 



LI 



I 1 



I L 



IS 



Co. 



■Co. 



Fig. 64. — Diagram showing 
the Relation of the Vertebral 
Spines to their Bodies and 
to the Origin of the Several 
Nerve-roots. (Gowers. ) 



The spinal cord, being a continua- 
tion of the brain, is surrounded by 
the same membranes. It is first en- 
veloped by the pia mater, which lies 
very closely to its surface, and sends 
small branches into the substance of 
the cord, and also passes along the 
nerve-roots, forming the immediate 
sheath of these roots. The arach- 
noid, like the arachnoid of the brain, 
is a thin membrane separated from 
the pia by minute threads of connec- 
tive tissue and serous fluid. Fluid is 
also present between the arachnoid 
and the dura mater, which differs 
from the dura mater around the brain 



ANATOMY OF THE SPINAL CORD. 



265 



by not remaining in immediate contact with the bony parts, 
but is separated from the latter by fatty tissue and a plexus 
of large veins. The dura mater also sends a prolongation 
along each nerve-root and helps to make up its sheath. 
The spinal cord is, as we see, safely suspended within the 
spinal canal, and not quite as much exposed to the effect 
of slight traumatic injuries as is the brain. 

In the spinal cord the gray and the white matter occupy 
relatively different positions from that in the brain. In the 
brain the gray matter invests 
the white matter, whereas in 
the spinal cord the white mat- 
ter completely encircles the 
gray, except at the two points 
at which the roots emerge from 
or enter into the spinal cord. 
The H-shaped figure represent- 
ing the gray matter of the cord 
is so well known that it scarce- 
ly needs accurate description. 
Its size varies according to the 
importance and number of the 
parts connected with the re- 
spective spinal-cord segments. 
Thus we find that there is more 
gray matter in the cervical and 
lumbar portions of the spinal 
cord, while there is least in the 
dorsal portions. The anterior 
or ventral portion of the gray 
matter of the spinal cord undergoes far greater variation in 
the different segments than the central and posterior por- 
tions do. This anterior variation is dependent very largely 
upon the number of cells which the gray matter harbors at 
each level. This has been well brought out bv the annexed 
figures, taken from Collins's article, who shows that the dif- 
ferences in the configuration of the anterior portion is due 
to the fact that the number of cells in the fifth is more than 
twice the number in the fourth cervical segment. 

The gray matter may be conveniently divided into the 




Fig. 65.— Outlines of IV and V Cer- 
vical Segments. (After Collins.) 



266 



THE NERVOUS DISEASES OF CHILDREN. 



anterior and posterior horns, with a layer of intermediate 
gray substance between them. In the dorsal region, a spe- 
cial projection of gray matter extends into the lateral col- 
umns and is known as the lateral horn. The gray matter 
consists of a groundwork of fine connective tissue in which 

A.C.(M.Gr.) 

■A.C.(La.Gr) 



■ACXElCti 




Fig. 66.— Cross-section of Sixth Cervical Segment of a Boy, Two Years Old. (After 
Waldeyer.) A. C. { M. Gr.), anterior cells (median group) ; A. C. {la. Gr.), anterior 
cells (antero-lateral group) ; A. C. {PI. Gr.), anterior cells (postero-lateral group) ; 
Sc. C, scattered cells ; C. C„ cells of column of Clarke ; M. C., middle cells ; L. C, 
lateral horn cells; P. H., posterior horn ; S3. Rol., substantia Rolandi ; i., cell of 
substantia Rolandi, with caudate process ; R. C, Rolandic cells ; Str. Zon. , stratum 
zonale ; L. M. Z. , Lissauer's marginal zone ; Stibp. L., subpial layer; P. R. , posterior 
root ; P., prolongation of pia ; a., artery ; C. B., column of Burdach ; C. G., column 
of Goll. 



small medullated nerve-fibres are embedded, as well as in- 
numerable small nerve-fibrils which arise in part from the 
branching cells of the gray matter, and in part represent a 
minute subdivision of the nerve-fibres that enter the cord 



ANATOMY OF THE SPINAL CORD. 



267 




Fig. 67. — Diagram showing the Probable Relations of Some of the Principal Cells and 
Fibres of the Cerebro-spinal System to One Another. (Schafer. ) 1, a cell of the 
cortex cerebri ; 2, its axis-cylinder or nerve process, passing down in the pyramidal 
tract and giving off collaterals, some of which, 3, 3, end in arborizations around cells 
of the anterior horn, the main fibre having a similar ending at 4 ; call. , sir., collateral 
fibres to corpus callosum and corpus striatum ; 5, axis-cylinder process of ganglion 
cell, passing to form a terminal arborization (end-brush) in the end-plate of a mus- 
cular fibre, m. ; 6, cell of spinal ganglion, with bifurcating axis-cylinder process, one 
branch, 7, ending in the skin, the other branch bifurcating after entering the cord (at S), 
its divisions passing upward and downward (the latter for a short distance only') ; 
9, ending of descending branch in a terminal arborization around a posterior horn 
cell, the axis-cylinder process of which again ends in similar fashion around a gan- 
glion cell of anterior horn ; 10, 11, other collaterals passing to ganglion cell and cell 
of Clarke's column respectively ; 14, 14, axis-cylinder processes of cells of posterior 
horns passing to form an end-brush around a ganglion cell ; 15, a fibre of cerebellar 
tract passing up toward a cerebellar cell ; 16, axis-cylinder process of this cell con- 
necting, giving off collaterals to anterior-horn cells of spinal cord ; 17, axis-cylinder 
process passing as a fibre of the fillet and ending near one of the cerebral cells : iS, 
axis-cylinder process of this cell, forming an end-brush around the pyramidal cell, 1. 



268 THE NERVOUS DISEASES OF CHILDREN. 

and subdivide either at the same level at which they enter 
or at higher or lower levels. The most important con- 
stituents of the gray matter are the nerve-cells imbedded 
in it. Those in the anterior portion of the gray matter are 
typical ganglion cells containing a large nucleus with much 
pigment and a number of different processes. In addition 
to these ganglion cells we also find a large number of 
smaller cells, which vary much in shape, but are character- 
ized by the absence of distinct processes. 

The ganglion cells, which may be considered the highest type of nerve-cell, 
have two distinct kinds of processes, a finer or axis-cylinder process, and 
stouter or protoplasmic processes. The axis-cylinder process divides into a 
number of terminal branches. These terminal fibres divide up again and end 
in minute fibrils (end-brush) ; the protoplasmic process divides up into a 
large number of fibrils, which either come into contact with one another or 
embrace the terminal fibrils of other cells of the gray matter. An actual 
transition from these into nerve-tracts has not been observed. The entire 
nervous system is made up of a series of units consisting of a ganglion cell, 
its dendritic processes, and the axis-cylinder process with its end-brush. 
For this unit Waldeyer has proposed the term " Neuron." 

The ganglion cells of the anterior cornua are divided into distinct groups. 
The position of these groups can be studied best on a cross-section (Fig. 66). 
These cell groups have very evident relations to the nerve branches issuing 
from the respective segments. 

Small cells are scattered in the intermediate gray matter, and a group of 
cells occupies the intermediate process, for which Gowers has proposed the 
name of intermediate group. Another group of cells, found chiefly in the 
lower dorsal and upper lumbar cord at the inner part of the neck of the pos- 
terior horn, has attained special distinction on account of the important part 
which these cells play in many of the diseases of the spinal cord. They 
were termed by Lockhart Clarke the posterior vesicular column, and are now 
more frequently described as the column of Clarke. 

In contradistinction to the general spongy portion which forms the main 
part of the gray matter, there is a distinct gelatinous substance which forms a 
cap, as it were, on the posterior horn. This is spoken of as the gray sub- 
stance of Rolando or the caput cornu posterioris. Through this substance 
many of the fibres pass which come from the posterior roots, thus dividing it 
up into distinct parts, and it also contains some small ganglion cells, some of 
which undoubtedly connect with the ramifications of the posterior fibres, 
while others are constituent parts of the neuroglia tissue. 

The white matter of the spinal cord would seem to con- 
stitute a continuous and homogeneous envelope around the 
gray figure, except at the two points at which it is broken 






ANATOMY OF THE SPINAL CORD. 269 

by the passage through it of the anterior and posterior 
roots; but the study of the development of the cord and of 
the degeneration which occurs in disease, has shown that 
this white matter contains a number of different systems of 
fibres which are continuous in a longitudinal direction and 
which have the function of simple conducting tracts ; 
some conducting impulses upward, others conducting im- 
pulses in a centrifugal direction and playing the part of 
mediating agents between higher and lower levels. 

The first discovery of the component parts of the white matter we owe 
to Tiirck, who determined that the nerve-fibres in the white substance of the 
cord undergo secondary degener- 
ation when severed from their 
cells of origin, just as the white 
fibres in other parts of the ner- 
vous system suffer. A further 
great advance in the study of 
spinal -cord tracts was effected 
by Flechsig's discovery that the 
nerve-fibres of different tracts 
acquire their medullary sheaths 
at different periods of develop- 
ment. Fibres whose medullary 
sheath is developed at about the FlG - 68.— Descending Degeneration of Direct 

same period of time evidently and Crossed p X ramidal Trac ts after a Lesion 
... , , in the Internal Capsule ; Slight Affection of 

belong together and are parts of columns of Goll due to Other Causes. 
one system. 

To the methods of Tiirck and Flechsig we may add the atrophy method 
of v. Gudden, which was based upon the discovery that parts that are in 
disuse undergo atrophy. If, therefore, an animal, otherwise healthy, were 
deprived at birth of important functions, the fibres connected with that func- 
tion would become atrophied. Such atrophied fibres constitute a continuous 
tract. Although this last method has been of much more sen-ice in the 
study of brain tracts, its principles may be and have been applied to the 
elucidation of the various systems of the spinal cord. 

As a result of these various methods of investigation we 
now know that the white substance of the cord may be sub- 
divided as follows: First, the anterior columns between the 
two anterior horns of the gray matter ; second, the lateral 
columns between the apex of the anterior horns and the 
posterior gray matter ; third, the posterior columns between 
the two posterior horns. All of these columns bear further 




270 



THE NERVOUS DISEASES OF CHILDREN. 







subdivision. Inasmuch as fibres always de- 
generate, according to the Wallerian law, 
from the lesion in the direction in which the 
impulses are carried, we are able to distin- 
guish between tracts that degenerate down- 
ward and those that degenerate upward. 
The former will be motor in function, the 
latter sensory. Two tracts have been found 
on the cross-section of the cord which invari- 
ably degenerate downward. The larger one 
of these tracts is situated in the lateral col- 
umns of the cord (5) ;* the smaller one (1) in 
the median portion of the anterior columns. 
These are the pyramidal tracts which are the 
continuation of the motor-fibres coming from 
each half of the brain, and since these fibres 
pass through the pyramids of the medulla, 
the tracts are spoken of as the pyramidal 
tracts. The larger or crossed pyramidal tract 
in the lateral columns contains the decus- 
sated fibres from the opposite half of the 
brain ; the smaller or direct pyramidal tract 
contains the fibres that have not decussated 
above. As a rule, the direct pyramidal tract 
has but little practical importance, although 
Flechsig maintains that in exceptional cases 
one-half or even less of the fibres decussate, 
but the rule is that at least from seventy to 
eighty per cent, of all motor-fibres undergo 
decussation, and must therefore be traced to 
the lateral columns of the cord. The crossed 
pyramidal tract lies posteriorly to the bulg- 
ing part of the H-shaped figure, but is sepa- 
rated from the surface by a small layer of 
fibres which constitute the direct cerebellar 
tract (6). The crossed pyramidal tract is larg- 
est in the cervical region. This is natural 
enough, since it gives off fibres at each level 
to the parts represented in a given segment. 

* The numbers on this and the following pages refer to Plate I. , Fig. 2. 







Fig. 69. — Seconda- 
ry Degeneration 
following a Le- 
sion in the Left 
Cerebral Hemi- 
sphere. (After 
Erb.) 



tic. I.— Sensory Tract, a, p, cells of spinal ganglia, one fibre,/, forming part of sensory nerve, 
the other fibre, c, entering a posterior root, fibres of the latter dividing into ascending and descend- 
ing (i, 2, 3, 4) branches ; of the ascending branches, some (4) terminate with " end-brushes " 
in the nucleus cuneatus, and nucleus gracilis ; col, collateral fibres entering gray matter • 8, 
fibres forming anterior ground bundle ; 5, 6, fibres forming lateral ground bundle ; 10, fibres 
forming Gowers's tract ; 7. fibres forming direct cerebellar tract. 




Frc;. II —r.a., anterior root ; /-./., posterior root ; I.R., Lissauer's marginal /one ; 1, direct pyram- 
idal tract; 2, anterior ground bundle ; 3, lateral ground bundle ; 4. Gowers's anterolateral tract ; 
5, crossed pyramidal tract ; 6, direct cerebellar tract ; 7, column of Burdach ; S, column of Goll ; 
9, posterior longitudinal septum; 10, anterior longitudinal fissure ; 11. anterior median group 
of cells; 12. postero-lateral group; 13. column of Clarke. 

Fir,. III. — Relation of motor tract to nuclei of cranial nerves. (After Flatau.) 



ANATOMY OF THE SPINAL CORD. 27 1 

While the cervical region, therefore, contains fibres for the 
arms, the trunk, and the legs, in the lumbar region it con- 
tains those for the legs only. The fibres for the legs evi- 
dently occupy the posterior portion of the tract in the cer- 
vical region, and as a result the pyramidal tract narrows 
down in this direction as it reaches lower and lower in 
the cord. The connection between the pyramidal tracts 
and the gray matter will be considered when we review 
the entire cross-section of the cord. 

The direct pyramidal tract passes down in the ventral portion of the cord 
and lies immediately adjoining the anterior fissure. It is most prominent in 
the cervical and dorsal regions, but there is no trace of it in the lumbar en- 
largement. It is generally supposed to end in the mid-dorsal region, except 
in those cases in which there is some anomalous decussation. The majority 
of the fibres evidently pass into the anterior commissure of the gray matter 
and to the opposite side of the cord, thus accomplishing very much the same 
that the larger body of fibres do ; we can understand therefore why so few 
symptoms are ever attributable to this direct pyramidal tract. A few fibres 
degenerating downward pass through the antero-lateral column, in that part 
that lies between the direct pyramidal tract and the direct cerebellar tract ; 
but these are of comparatively little importance and probably belong to the 
crossed pyramidal tract, though Foster has given them the special designation 
of descending antero-lateral tract. 

The study of the fibres which degenerate upward has led 
to an accurate localization of the sensory divisions of the 
cord ; chief among these are the posterior columns. The 
posterior columns of each side are divided by a narrow sep- 
tum ; they can be separated into two main divisions — first, a 
smaller postero-median column or column of Goll (8), and a 
broader postero-external column, or column of Burdach (7). 
That part of the column of Burdach which is situated next 
to the posterior horn is called the posterior root zone, and 
has acquired a special distinction of late through the fact 
that it contains the fibres of Lissauer's tract which degen- 
erate early in cases of tabes. The postero-median column 
is evidently the direct continuation upward of the fibres 
entering from the lowest level of the cord, as can best be 
seen in studying an ascending degeneration after a lesion 
of the cauda equina. 

The column of Goll is an extremely narrow, scarcely perceptible, tract in 
the lumbar portion of the cord, frequently not even touching the posterior 



272 THE NERVOUS DISEASES OF CHILDREN. 

commissure or the periphery of the cord. It grows in size as it proceeds up- 
ward in the cord, remains wedge-shaped in form, but its apex grows broader 
as it approaches the cervical region. In this region it does not quite attain to 
the commissure, and leaves a small crescent-shaped field which evidently has 
a special significance. 

The column of Burdach, or postero-external column, consists chiefly of short 
vertical fibres, and is made up mainly of fibres which pass upward and in- 
ward to the' median column or pass back into the gray matter. The fibres of 
this column soon connect with nerve-cells, and for this reason do not degen- 
erate for any great distance above the seat of the lesion. The result is that 
in any lesion of the spinal cord an ascending degeneration will be restricted 
chiefly to the postero-median column, and will not be very marked in the 
postero-lateral column. This ends above in the so-called nucleus cuneatus, 
while the column of Goll ends in the nucleus gracilis (Fig. 71). These nuclei 
are connected by fibres passing chiefly through the lemniscus to the opposite 
cerebral cortex, ending in the motor area or parts immediately adjacent to it. 

Of the direct cerebellar tract (6) we need merely say that it also degener- 
ates upward. It does not extend below the level of the first lumbar nerve. 
The tract increases in size from below upward, and must therefore receive 
fibres throughout its course ; the majority of them evidently enter the cord 
at the level of the lowest dorsal and first lumbar nerves. These fibres 
pass through the lateral column into the gray matter, and are probably con- 
nected with the posterior vesicular column, to which they are related beyond 
a doubt, as these cells have also been found degenerated in cases in which 
the direct cerebellar tract has been atrophied. This direct cerebellar tract 
passes through the restiform body into the cerebellum. 

In addition to the tract just described there is another, a small, narrow 
tract (4), which was discovered by Gowers and first described by him in 1879. 
The tract occupies an irregular area in front of the pyramidal and cerebellar 
tracts, and degenerates upward throughout the cord. According to his de- 
scription it extends across the lateral column as a band which fills up the 
angle between the pyramidal and cerebellar tracts, and it reaches the surface 
of the cord in front of the latter tract, nearly on a level with the anterior 
commissure ; it then extends forward in the periphery of the anterior column 
almost to the anterior median fissure, and up to the direct pyramidal tract 
when this exists. Bechterew has found that this tract undergoes develop- 
ment at a different period from the rest of the lateral column. As it de- 
generates upward it is unquestionably sensory in function, but its fibres do 
not degenerate when the nerve-roots are divided, whence it is probable that 
its fibres arise from cells in which the root-fibres end. We may safely con- 
sider it one of the sensory tracts connecting the spinal cord with the hemi- 
spheres, but its exact functions have not been determined. 

Further subdivisions of the white matter will, no doubt, be necessary as 
our knowledge of the anatomy of the cord increases. For the present we 
are left with a large mass of white fibres which Flechsig terms the anterior 
ground fibres, to which we cannot as yet assign a positive function. They 
do not degenerate through any considerable extent of the cord, and as some 



ANATOMY OF THE SPINAL CORD. 



273 



of the fibres pass to the anterior commissure, in which a marked decussation 
takes place, the fibres in these anterior ground bundles may serve to connect 
the two halves of the cord. 

The commissure lies between the bottom of the anterior fissure and the 
posterior columns. It consists of an anterior white portion and a posterior 
gray portion. The white portion is largest in the lumbar region, and con- 
tains a large number of medullated fibres which cross from the anterior half 
of one side of the commissure to the posterior half on the other side. The 
anterior commissure also contains fibres that connect the anterior white col- 




Fig. 70. — Diagrammatic Cross-section of Spinal Cord, showing the Course of the More 
Important Tracts within the Cord. (Slightly modified, after Edinger.) 

umns of one side with the anterior cornu of the other. Other fibres pass 
through the commissure from one anterior pyramidal tract through the in- 
termediate gray substance into the lateral column, thus constituting a con- 
nection which is practically equivalent to a decussation (Fig. 70). 

The gray portion of the commissure contains very often medullated nerve- 
fibres, a number of which pass into the posterior horn of each side. These 
are continuous with the fibres in the posterior horns, and it is, therefore, 
likely that much of the sensory decussation in the cord is effected by these 
fibres passing through the gray commissure. 

It has been customary to think of the structure of the 
spinal cord as represented by the appearance on cross-sec- 

iS 



274 THE NERVOUS DISEASES OF CHILDREN. 

tion. This has been done to the detriment of the more 
fitting conception of the spinal cord as an organ of longi- 
tudinal extent connecting the brain with the periphery. 
The irregularly shaped gray matter, for instance, is not, 
properly speaking, H-shaped, but should be conceived of as 
a fluted column running up and down through the centre 
of a large pillar. The white matter practically surrounds 
this inner gray substance and fills out all the indentations 
in such fashion that the spinal cord becomes a long cylin- 
drical mass. From this cylindrical mass the connections 
are made with the periphery by means of the anterior and 
posterior root-fibres. 

Taking up now the course of the anterior and posterior 
root-fibres, we shall find that the course of many of the 
component parts has been well established by recent re- 
searches. Among the authors who have contributed most 
successfully to this special subject, we must mention Golgi, 
Ramon y Cajal, Lenhossek, Kolliker, Edinger, Obersteiner, 
and Waldeyer. The following special connections between 
the anterior root -fibres and spinal-cord structures have 
been made out. First, fibres connecting directly with the 
large ganglion cells in the anterior horns, some connecting 
with the median group, some indirectly with the lateral 
and postero-lateral groups of cells (Figs. 66, 70). Second, 
fibres penetrating the gray matter for some distance and 
ending in its dorsal half. Third, fibres entering at one seg- 
ment and connecting with ganglion cells at higher and 
lower levels. Fourth, another set of fibres passes over to 
the opposite half of the spinal cord, through the anterior 
white commissure, and end in some of the spindle-shaped 
cells near the inner margin of the gray matter. Whether 
any anterior root-fibres pass directly through the gray mat- 
ter connecting with the longitudinal fibres of the white 
columns has not been positively established. It is, indeed, 
probable that the connection between the pj^ramidal tracts 
and the anterior horns, as important a connection as any, 
is made through terminal arborizations (3, 3, Fig. 67). 

The course of the posterior root-fibres has also been unravelled within re- 
cent years, although it is more than probable that we are but at the begin* 
ning of our knowledge regarding the exact course and functions of these 






ANATOMY OF THE SPINAL CORD. 



275 



various sets of fibres (Fig. 70, 1-5). First, the laterally situated root-fibres im- 
mediately upon entering the cord ascend in a longitudinal direction, and as seen 
on cross-section give rise to a distinct zone of fibres, which has been de- 
scribed by Lissauer, and is now known as the tract of Lissauer. After 
ascending for a short distance these nerve-fibres turn into the gray matter, 
the substantia gelatinosa, and for this reason the column of Lissauer does 
not increase very much in the direction 
toward the brain (5). Second, another 
set of fibres adjoining these pass first 
through the lateral portion of the col- 
umn of Burdach and enter the sub- 
stantia gelatinosa. From here they 
pass into the substantia spongiosa, 
some of the fibres passing in a ventral 
direction and others taking a longitudi- 
nal direction upward and downward. 
It is probable that all of these fibres 
leave the posterior horns sooner or 
later ; some of them ending, however, 
in nerve-cells situated in the posterior 
horns. Pal has claimed that a few of 
these fibres can be traced in their course 
through the anterior gray substance to 
the anterior large cells (4), and Edinger 
states that some of them pass through 
the anterior commissure into the an- 
terior columns of the opposite side. 
Others, again, leaving the ventral por- 
tion of the posterior horns, pass into 
the adjacent white matter and remain 
in that part of the white substance 
known as the lateral limiting layer (3). 
Of the more median portion of the 
posterior root-fibres many enter the 
column of Burdach, make a broad 
sweep, and then take a longitudinal 
direction (2). Some of these fibres can 
be traced through the small cells situ- 
ated in the posterior gray commissure. 
Another set of fibres pass directly to 

the columns of Clarke, and through these are connected with the cerebellar 
tract. Another considerable portion of the posterior root-fibres passes di- 
rectly into the posterior columns and ascends, without crossing over to the 
other side, to the nucleus gracilis, but passes through the sensory decussation 
in the brain (1). 

There is one marked distinction between the anterior root-fibres and those 
making up the posterior roots. The nutritive cells of the former are the gan- 




FlG. 71. — Course of Sensory Fibres from 
the Posterior Roots to the Medulla 
Oblongata. (Edinger.) 



276 



THE NERVOUS DISEASES OF CHILDREN. 



glion cells of the anterior horns, whereas the spinal ganglion situated outside 
of the spinal-cord substance exercises the same function relative to the pos- 
terior fibres. Recent investigations have furthermore shown that these pos- 
terior root-fibres divide in Y-shaped fashion into an ascending and descend- 
ing branch. Both these branches enter the gray matter, and here again 
subdivide, while taking a longitudinal course, into a number of fibres, each 
one again giving off collateral branches, and thus establishing a neural net- 
work. 

These details of structure would possess little interest if they did not help 
to disclose a functional relation of the different parts of the spinal cord. By 
means of these anatomical studies we can understand, for instance, that fibres 
ending in the gray cells of the anterior horns are specially intrusted with mere 
nutritional functions for the nerve and muscles with which they are connected ; 

that certain other cells may be 
connected with the electrical 
conditions of nerves and mus- 
cles ; that those fibres that 
either pass directly into the 
posterior portion of the gray 
matter, or make indirect con- 
nections with it, are part of the 
reflex arc passing through a 
given segment ; while fibres es- 
tablishing a connection between 
the anterior gray matter and 
the lateral columns are in all 
probability those which trans- 
mit motor impulses. Just so 
the various portions of the pos- 
terior root - bundles, as they 
divide up in the cord, in all 
probability carry special func- 
tions with them. It is not a 
great stretch of the imagination 
to suppose that tactile sensa- 
tion and the sensory impulses 
by which reflex action is excited 
pass through the lateral series of fibres, whereas those fibres connecting with 
the columns of Clarke in all probability have to do with the function of co- 
ordination and with the transmission of visceral sensations. Other sets of 
fibres again must be engaged in the transmission of muscular sense, of pain, 
and of tactile sensation; but our studies are not yet sufficiently advanced 
to point out the exact groups of fibres intrusted with each special func- 
tion. 

Having traced the course of these fibres in a spinal-cord segment, it will 
be well to refer to the efforts made by Thorburn, Head, Starr, and others to 
prove the exact distribution of function in the spinal cord, showing the main 




Fig. 72. — Division of Sensory Root-fibres — Hu- 
man Embryo of Six Months. (Golgi.) 



LOCALIZATION IN THE SPINAL CORD. 



277 



functions depending upon the individual segments. As the best summary 
of spinal localization I give Starr's table, as he has kindly revised it for me, 
with the additions and corrections suggested up to date. 

Localization of the Functions of the Segments of the Spinal Cord. 



Segment. 


1 

Muscles. 


Reflex. 


Sensation. 


II. and 


Sterno-mastoid. 


Hypochondrium (?). 


Back of head to 


III. C. 


Trapezius. 


Sudden inspiration pro- 


vertex. 




Scaleni and neck. 


duced by sudden press- 


Neck. 




Diaphragm. 


ure beneath the lower 
border of ribs. 




IV. C. 


Diaphragm. 


Pupil. 4th to 7th cervical. 


Neck. 




Deltoid. 


Dilatation of the pupil 


Upper shoulder. 




Biceps. 


produced by irritation 


Outer arm. 




Coraco-brachialis. 


of neck. 






Supinator longus. 








Rhomboid. 








Supra- and infra-spinatus. 






V. c. 


Deltoid. 


Scapular. 


Back of shoulder 




Biceps. 


5th crevical to 1st dorsal. 


and arm. 




Coraco-brachialis. 


Irritation of skin over the 


Outer side of arm 




Brachialis anticus. 


scapula produces con- 


and forearm, 




Supinator longus. 


traction of the scapular 


front and back. 




Supinator brevis. • 


muscles. 






Rhomboid. 


Supinator longus. 






Teres minor. 


Tapping its tendon i n 






Pectoralis (clavicular 


wrist produces flexion 






part). 


of forearm. 






Serratus magnus. 






VI. c. 


Brachialus anticus. 


Triceps. 


Outer side of fore- 




Serratus magnus. 


5th to 6th cervical. 


arm, front and 




Triceps. 


Tapping elbow tendon- 


back. 




Extensors of wrist and 


produces extension of 


Outer half of 




fingers. 


forearm. 


hand. 




Pronators. 


Posterior wrist. 
6th to 8th cervical. 
Tapping tendons causes 
extension of hand. 




VII. c. 


Triceps (long head). 


Anterior wrist. 


Inner side and 




Extensors of wrist and 


7th to 8th cervical. 


back of arm and 




fingers. 


Tapping anterior tendons 


forearm. 




Pronators of wrist. 


causes flexion of wrist. 


Outer half of the 




Flexors of wrist. 


Palmar. 7th cervical to 


hand. 




Subscapular. 


1st dorsal. 






Pectoralis (costal part). 


Stroking palm causes 






Latissimus dorsi. 


closure of fingers. 






Teres major. 






VIII. c. 


Flexors of wrist and 




Forearm and 




fingers. 




hand, inner half. 




Intrinsic muscles of hand. 







278 



THE NERVOUS DISEASES OF CHILDREN. 



Localization of the Functions of the Segments of the Spinal Cord. — 

Continued. 



Segment. 


Muscles. 


Reflex. 


Sensation. 


I. D. 


Extensors of thumb. 
Intrinsic hand muscles. 
Thenar and hypothenar 
eminences. 




Forearm, inner 

half. 
Ulnar distribution 

to hand. 


II. to 
XII. D. 


Muscles of back and 

abdomen. 
Erectores spinas. 


Epigastric. 4th to 7th 

dorsal. 
Tickling mammaryregion 

causes retraction of the 

epigastrium. 
Abdominal. 7th to nth 

dorsal. 
Stroking side of abdomen 

causes retraction f 

belly. 


Skin of chest and 
abdomen, i n 
horizontal 
bands running 
around the 
body. 


I. L. 


Ilio-psoas. 
Sartorius. 
Muscles of abdomen. 


Cremasteric. 1st to 3d 

lumbar. 
Stroking inner thigh 

causes retraction f 

scrotum. 


Skin over groin 
and front of 
scrotum. 

Over back above 
buttocks. 


II. L. 


Ilio-psoas. Sartorius. 
Quadriceps femoris. 
Flexors of knee. 


Patella tendon. 
Striking tendon causes 
extension of leg. 


Front of thigh. 


III. L. 


Quadriceps femoris. 
Flexors of knee. 
Inner rotators of thigh. 
Abductors of thigh. 




Front and outer 
side of thigh. 


IV. L. 


Abductors of thigh. 
Adductors of thigh. 
Tibialis anticus. 


Gluteal. 

4th to 5th lumbar. 

Stroking buttock causes 
dimpling in fold of but- 
tock. 


Outer side of leg. 

Inner side of thigh 

and leg to ankle. 

Inner side offoot. 


V. L. 


Outward rotators of thigh. 
Flexors of ankle. 
Extensors of toes. 




Back of thigh, back 
and outer part of 
leg and of foot. 


I. to II. 

S. 


Flexors of ankle. 

Long flexor of toes. 

Peronei. 

Intrinsic muscles of foot. 


Plantar. 

Tickling sole offoot 
causes flexion of toes 
and retraction of leg. 


Back of thigh and 
outer side f 
foot. 

Buttocks. 


III. to 
V. S. 


Perineal muscles. 


Foot reflex. Achilles 
tendon. 

Over-extension of f t 
causes rapid flexion ; 
ankle-clonus. 

Bladder and rectal cen- 
tres. 


Skin over sacrum, 
anus, perineum, 
genital s, and 
lower part of 
buttocks. 



FUNCTIONS OF THE SPINAL CORD. 279 

Referring once more to the cross-section of the cord we 
can state that the anterior gray matter has a fourfold func- 
tion : First, it transmits motion ; second, it preserves the 
normal electrical condition of the nerves and muscles gov- 
erned by the respective segment ; third, it keeps up the 
normal tonus of muscles ; and fourth, it contains an integral 
part of the reflex arc. The result is that in disease of the 
gray matter these four functions are disturbed ; we have, 
therefore, paralysis, atrophy, altered electrical conditions 
(some form of the reaction of degeneration), and loss of re- 
flexes. The special function of the posterior gray matter 
would seem to be that of providing for the proper trans- 
mission of sensory impulses and of maintaining the health 
of the posterior root-fibres. The functions of these pos- 
terior root-fibres and of the posterior horns is to trans- 
mit the various forms of sensation, namely, the sensation 
of touch, of pain, of heat and cold, and the sensation of 
muscular innervation. They also contain the sensory fibres 
which go to make up the reflex arc. A lesion in these parts, 
if complete, would therefore cause a disturbance of every 
form of sensation, interference with the visceral reflexes 
and with the deep tendinous reflexes. The majority of 
diseases do not, however, involve this entire region, that 
is, both the posterior columns and the posterior horns, and 
for that reason in many diseases, such as tabes for instance, 
only some of the functions of these parts are disturbed, 
while others may remain entirely intact. 

The lateral columns of the cord have unquestionably a 
variety of functions, but only those which can be attributed 
directly to the pyramidal tracts in the lateral columns are 
sufficiently well known to demand consideration. Bv far 
the most important function of the pyramidal tracts is the 
transmission of motor impulses from a higher to a lower 
level. As was said before, they are the direct continuation 
of the pyramidal tracts in the brain, each pyramidal tract 
being chiefly connected with the motor tract in the opposite 
half of the brain. Disease of the pyramidal tracts, there- 
fore, implies paralysis, but since the disease of the lateral 
columns is most frequently secondary, the paralysis is not 
so much the result of the lesion in the lateral columns as it 



280 THE NERVOUS DISEASES OF CHILDREN. 

is of the lesion higher up which has given rise to the de- 
generation in these columns. When paralysis accompanies 
disease of the lateral columns it is associated with two 
symptoms which need further consideration, and which are 
due directly to the diseased fibres in the spinal cord. 

The first of these symptoms is the spastic contracture of the paralyzed 
parts. Various explanations have been given for this phenomenon. The 
most plausible of these is that advanced by Strauss, in 1875, which has re- 
cently received the qualified approval of Marie. According to this explanation 
the contracture is an exaggeration of the normal muscular tonus, and this 
exaggeration is due to the exciting effect which the diseased fibres in the 
lateral columns have upon the neighboring ganglion cells ; in other words, a 
hypernormal tonus is brought about which is due to an irritation of the large 
ganglion cells of the anterior horns. There is one serious objection, how- 
ever, to this view, and that is, that if diseased fibres constitute a source of 
permanent irritation it would be difficult to explain why this condition of 
spasmodic contracture occurs in cases in which there is a defective develop- 
ment of the lateral columns without any indication whatever of disease in 
these parts. This happens in cases of infantile palsies in which there is a 
congenital defect of the motor region of the brain and of the entire motor 
tract throughout the spinal cord. I have myself had occasion to examine the 
brain and cord of a child born with this defect, in whom the spasmodic con- 
tractures of both legs were very extreme. For such cases we must seek a 
different explanation, and while I believe that Strauss's explanation is sufficient 
for many cases, I am inclined to think that we must call into use the doctrine 
of cerebral inhibition and make a lack of such inhibition responsible for con- 
tinued over-action of the ganglion cells. I do not see any sufficient reason 
for insisting that one set of phenomena shall invariably be due to a single 
cause. 

We must rely also upon the doctrine of cerebral inhibi 
tion for an explanation of the effect which disease of the 
lateral columns has upon the deep reflexes. It is well known 
that the deep reflexes are increased whenever there is a 
lesion in the lateral columns of the cord or in any higher 
part of the pyramidal tract. The theory that these increased 
reflexes are the result of constant irritation of the ganglion 
cells intrusted with this function is not satisfactory, for the 
reflexes are enormously increased in those cases in which 
the lateral columns are imperfectly developed or practically 
absent. Under normal conditions the reflexes are under 
cerebral control, and over-action of the ganglion cells is thus 
rendered impossible ; but let this cerebral inhibition be 



ANATOMY OF THE SPINAL CORD. 



28l 



wanting and the ganglion cells have things very much their 
own way and can over-act to the greatest possible extent ; 
hence the reflexes are increased. 



A fact worth mentioning in this connection is that the reflexes are fre- 
quently increased on both sides, even though the lesion in the brain or else- 
where be unilateral. In some 
cases of hemiplegia there may- 
be contracture on the sound 
side and the reflexes may be 
increased on both sides. Pi- 
tres has collected ten cases in 
which both pyramidal tracts of 
the lateral columns have been 
degenerated after unilateral le- 
sion in the brain. A satisfac- 
tory explanation of this condi- 
tion has not yet been given, 
but the probability is that there 
are connections between both 
crossed pyramidal tracts in the 
spinal cord and also between 
the direct pyramidal tract in 
the anterior columns and the 
crossed pyramidal tract on the 
same side of the spinal cord. 



THE BLOOD-SUPPLY OF THE 
SPINAL CORD. 




The chief arteries supplying 
the spinal cord are as follows : 
The anterior spinal, which is 
double at its upper end as it 
issues from the vertebrals, but 
its two branches unite below 
into one artery, which is rein- 
forced by small vessels that 
come off from the vertebral, 
intercostal, lumbar, and sacral 
arteries, and pass to the cord 



Fig. 73. — Blood Supply of Spinal Cord. (Dia- 
gram after Obersteiner and Schafer.) a. s.a., 
anterior spinal artery ; c. , a central arteriole ; 
a., an anastomosis branch uniting it with anoth- 
er arteriole of an adjacent segment ; CI. , branch 
to Clarke's column ; a. p./., artery of posterior 
fissure ; /. m. a., posterior mesial artery ; a. p. c, 
artery of posterior cornua ; p. s. a., branch of 
posterior spinal artery passing into gelatinous 
substance ; /., other peripheral or centripetal 
arterioles passing through white substance ot 
cord. 



along with the anterior roots. 

The posterior spinal arteries are also derived from the vertebrals, intercostal, 
and other arteries, and pass to the cord next to the posterior roots. The 
branches of these vessels are distributed in the pia matter surrounding the 
cord, communicating freely with one another by means of transverse anas- 



282 THE NERVOUS DISEASES OF CHILDREN. 

tomoses and sending vessels into the gray and white substance. These 
branches as they enter the cord practically form two systems, a centrifugal 
and a centripetal system. The centrifugal is made up of the central arteri- 
oles described by Ross, which pass from the anterior spinal artery into the 
artery of the median fissure (c) and enter the anterior commissure ; each 
branch here passes to the right or left and divides into smaller arteries and 
capillaries in the central gray matter. Branches also run up and down, 
intermingling with the branches that are given off above and below, and 
while distributed chiefly to the central gray matter, send off branches that 
enter the white matter. 

The centripetal arterioles are arranged in a convergent fashion, passing 
inward from the periphery. Some of them form small capillary loops, others 
go to make up large-meshed longitudinal plexuses ; the larger of the centrip- 
etal arteries pass into the gray matter and end in a net-work of capillaries 
and supply those parts which are not supplied by the centrifugal. No one 
part of the spinal cord is supplied exclusively by one set of arterioles, nor is 
the area of distribution of any one system limited exclusively to white or gray 
matter. As in the brain, however, so in the spinal cord, the arteries are prac- 
tically terminal arteries, each one having its own area of distribution and not 
communicating with areas supplied by other vessels. According to Kadyi 
there is considerable variation in the capillary blood-supply of any one area. 

The veins of the spinal cord are these : two median vessels, one accom- 
panying the anterior spinal artery and the other running along the posterior 
median fissure. Both these veins receive their blood from a large number of 
smaller branches, which in turn give off a number of smaller veins along the 
nerve-roots, which help to carry the blood off quickly. In the upper part of 
the cord the veins are connected with those of the cerebellum and pons, and 
empty into the venous sinuses around the foramen magnum. The anterior 
spinal veins receive their blood from innumerable small central veins, these 
collect the blood from the gray matter; the small peripheral veins which 
enter into the veins of the pia matter collect the blood from the capillaries to 
the white matter of the cord. 



BIBLIOGRAPHY. 

Adamkiewicz : Sitzungsberichte der Mathem. Classe der Kaiserl. Academie 
der Wissenschaften, vol. lxxxiv., 1881, and vol. lxxxviii., 1882. 

y Cajal (Ramon) : Anat. Anzeiger, 1890. Les nouvelles Idees sur la Struct- 
ure du Systeme nerveux. Paris, 1894. 

Collins : New York Medical Journal, 1894. 

Edinger : Lectures on Structure of the Central Nervous Organs. American 
translation, 1890. See also third German edition, 1892. 

Flechsig, P.: Neurologisches Centralbl., 1890. 

Golgi : Archives ital. de Biologie, vols. iii. and iv., 1883. Untersuchungen 
iiber den feineren Bau des Centralen und Peripherischen Nervensystems. 
Jena, 1894. 



ANATOMY OF THE SPINAL CORD. 283 

Gowers : Neurologisches Centralbl., 1886, Nos. 5 and 7. (On the antero- 

• lateral tract.) 
Homen : On Secondary Degeneration, etc., Virchow's Arch., vol. lxxxviii., 

1882. 
Kadyi : Ueber die Blutgefasse des menschlichen Ruckenmarkes. Lemberg, 

1889. 
Kahler : Article on Anatomy of Nervous System in Toldt's Gewebelehre, 

1888. 
Kolliker : Handbuch der Gewebelehre, etc., vol. ii. ; first half. Leipzig, 1893. 
Lenhossek : Beitrage zur Histologic des Nervensystems, etc. Wiesbaden, 

1894. 
Lissauer : On Course of the Fibres in the Posterior Horns of the Spinal Cord 

of Man ; Archiv f. Psychiatrie, vol. xvii., 1886. 
Obersteiner : Anatomy of the Central Nervous Organs : translated by Hill. 

London, 1890. 
Schafer and Thane : Quain's Anatomy, vol. iii., pt. 1, 1893 (the best book of 

reference in English). 
Singer : On Secondary Degeneration. Sitzungsberichte der Wiener Akade- 

mie. Bd. LXXXIV., 3 Abth., 1882. 
Spitzka: Journal of Comp. Medicine and Surgery, 1886. 
Striimpell : Zur Pathologie des Ruckenmarkes, Archiv f. Psychiatrie, vols. 

x. and xii. 
Waldeyer, W. : Deutsche Med. Wochenschrift, 1891. (Contains an excellent 

summary of the newer teachings.) Das Gorilla Ruckenmark. Berlin, 

1880. 



CHAPTER XV. 

ANEMIA AND HYPEREMIA OF THE SPINAL CORD. 

There is a reasonable doubt as to the existence of a dis- 
tinct form of disease due to an anaemic or hyperaemic con- 
dition of the brain, and there is still more doubt as to the ex- 
istence of any decided clinical condition due to the varying 
blood-supply of the spinal cord. The older authors were 
very honest in their search after definite symptoms due to 
these varying states of the vessels in the spinal cord and its 
meninges. The more recent writers, too, have followed in 
their footsteps, although every one seems to discuss this 
subject with an apology for attempting to establish a defi- 
nite series of symptoms, each one of which is rather vague, 
and may be due to a number of different causes. Monti, 
Gowers, Spitzka, and others have written rather elaborate 
articles on this subject, recognizing the difficulties in pre- 
senting the symptomatology of this condition, and yet feel- 
ing compelled to make definite statements regarding it. 
Anaemia and hyperaemia of the spinal cord represent mor- 
bid conditions which are often met with in connection with 
other processes. From a purely clinical point of view they 
are of little import. With the exception of the direct effects 
of concussion upon the spinal cord and its membranes, there 
is little occasion during life to make the diagnosis of anaemia 
or hyperaemia of the spinal cord, and the author is willing 
to state that in his own experience he has not found it nec- 
essary to make this diagnosis in any given case, although 
he may have had occasion, as others have had, to recognize 
the association of this condition with other more important 
morbid states and more important clinical symptoms. 

The diagnosis of anaemia and hyperaemia of the cord has 
very frequently been made to cover our ignorance regard- 
ing the exact morbid state ; or it has been made the substra- 



ANAEMIA AND HYPEREMIA OF THE SPINAL CORD. 285 

turn of conditions which we know to be purely functional, 
and which may possibly be due to altered blood-supply, but 
have not positively been proved to be due to this cause. No 
one at the present time will go the length to which Ham- 
mond went, some fifteen years ago, in attributing the ma- 
jority of cases of spinal irritation, so called, and of tem- 
porary palsies, to a condition of hyperaemia of the spinal 
cord. 

We may now enumerate the conditions under which anaemia or hyperaemia 
of the spinal cord may occur. Of the two conditions anaemia is by far the 
more serious one, and fortunately the rarer one. An absolute inhibition of 
blood-supply to the spinal cord is a condition that arises so rarely in life that 
the diagnosis need not be made ; moreover, it would lead to very rapid de- 
struction of the spinal cord. It is only in cases of a blocking up of the larger 
branches of the descending aorta that such condition occurs, and under these 
circumstances the part supplied by the aorta below the point of embolism 
would soon undergo complete necrosis. 

Transitory anaemia of the spinal cord may be the result of vasomotor 
spasm. This condition has been held responsible for many sensory symp- 
toms frequently occurring in connection with exhausting diseases and often 
independently of them. The sensations of tingling, of burning in the feet, of 
pins and needles, have been attributed by some writers to this cause ; but un- 
less such symptoms are symmetrical in their distribution, and unless there is 
some special reason to refer them to the spinal cord, I should be inclined to 
attribute them to peripheral vasomotor spasm, rather than to a similar con- 
dition affecting the blood-vessels of the spinal cord. At all events these same 
symptoms occur in connection with the lithaemic, rheumatic, and other states 
which we know affect the peripheral nerve system more frequently than they 
do the spinal cord. Gowers refers to the condition of " intermitting lame- 
ness " as possibly due to an anaemia of the cord, but Charcot's recent investi- 
gations of this subject make it much more likely that this condition is due to 
the brain than to the spinal cord. 

From the preceding we can infer that there are, after all, 
very few conditions to be attributed directly to an anaemia 
of the spinal cord, and we are practically compelled to ad- 
mit that the only conditions under which an anaemia of 
the spinal cord gives rise to a special set of symptoms are 
those associated with a general chlorosis or with the forms 
of pernicious anaemia. 

A number of authors — Lichtheim, Minnich, v. Noorden, and Nonne — have 
described a degeneration in the spinal cord in cases of pernicious anaemia.* In 

* Eisenlohr has directed attention to the changes in the spinal cord due to pro- 
found anaemia in association with gastric and intestinal disturbances. 



286 THE NERVOUS DISEASES OF CHILDREN. 

the majority of cases the posterior columns were chiefly affected ; in one 
half of them, the lateral and anterior white columns were also degenerated. 
The morbid process is, as a rule, most intense in the cervical region, and involves 
the dorsal and lumbar segments as well. It would be natural to suppose that 
the poorer blood-supply in the posterior half of the cord segment would 
account for the frequent affection of the posterior columns in pernicious anae- 
mia ; but there is some reason to think that in these cases not the anaemia, 
but toxic products circulating in the blood, are the chief causes of this spinal 
affection. 

The marked weakness of the legs observed in some anaem- 
ic persons, associated either with diminution of reflexes, or 
with an increase of reflex activity, is, in all probability, due 
to an imperfect blood-supply in the spinal cord. The same 
explanation will hold good for the extreme weakness after 
exhausting diseases, which is due probably to a weakness, in 
the one set of cases, of the ganglionic cells of the spinal 
cord, and in the other, of the white fibres of the cord. But 
even among these cases of extreme weakness after acute 
diseases, such as scarlet fever, pneumonia, and the like, the 
hyperesthesia and paresthesia existing at the time have led 
me to suspect that the condition is more probably due to an 
affection of the peripheral nerves than of the spinal cord. 

A condition of paralysis coming on after sudden and large 
hemorrhages may well be attributed to an anasmic condition 
of the cord, and the method of gradual recovery with im- 
proving health lends color to this view. But such sudden 
hemorrhages and the conditions resulting therefrom are far 
less frequent in children than in adults. 

Hyperemia of the spinal cord is still more difficult to di- 
agnosticate and to discuss as a separate clinical entity. It is 
undoubtedly true that a hyperemia of the cord, continuing 
independently of other morbid processes for a long period, 
never occurs. It is either a complication of some other 
morbid state of the cord, or is the first stage of an inflam- 
matory condition. Hyperemia due to stasis is observed 
frequently enough in the newborn, particularly after pro- 
tracted labor, and the occurrence of it can be understood 
quite readily in all those cases in which obstetrical ma- 
nipulations of one kind or another have produced persistent 
pressure upon the spinal column. It occurs also in those 
cases in which the child is born in an asphyxiated condition. 









ANJEMIA AND HYPEREMIA OF THE SPINAL CORD. 287 

It is present in the majority of cases of inflammation of 
the spinal cord (myelitis), in cases of periostitis and caries 
of the bone ; but in no instance does it occur as an indepen- 
dent disease, and it is doubtful whether West's assertion 
that it occurs as a condition complicating prolonged mas- 
turbation is correct, though the symptoms due to this cause 
closely resemble those generally regarded as symptoms of 
spinal irritation, and these latter symptoms have frequently 
been attributed to a hyperaemic condition. Hasse has 
maintained that hyperasmia of the spinal cord occurs in 
febrile diseases, and particularly in septic forms of diphthe- 
ria, in scarlatina, hemorrhagic variola, in cholera, typhus, 
etc. 

It would scarcely be worth our while to consider the 
symptomatology of hypersemia of the cord if this condition 
did not frequently precede a condition of true inflammation, 
and for that reason it is of some importance to recognize the 
early symptoms. Among these we might class a sensa- 
tion of heaviness in the legs, gradually increasing to de- 
cided weakness, sensations of pain and tingling, sometimes 
of heat and cold in the extremities ; but these symptoms 
should be at least symmetrically developed if they are to 
point to the spinal cord rather than to the peripheral 
nerves. Objective sensation is rarely disturbed, and reflex 
activity may be in some instances slightly diminished, in oth- 
ers slightly increased. Part or the whole of the spinal col- 
umn is, as a rule, slightly sensitive to touch, but not nearly 
as sensitive as in those cases in which there is distinct pe- 
riostitis or caries. There is no absolute paralysis of the 
sphincters of the bladder and the rectum ; but a slight irri- 
tability and hasty micturition in particular may occasionally 
be noticed. 

The very cases which have been reported and cited by various authors as 
typical of this condition seem to me to throw greatest doubt upon the actual 
existence of this state. Thus Monti quotes from West the case of a boy. three 
and half years of age, whose general health was affected, who showed a cer- 
tain awkwardness in gait which gradually increased until the boy became 
thoroughly paralyzed. The loss of motion was said to have been entirely 
out of proportion to the general condition of the boy's health. The habit of 
masturbation was discovered, and it is claimed that all the symptoms disap- 
peared very soon after the habit had been stopped. Why in this ease a eon- 



288 THE NERVOUS DISEASES OF CHILDREN. 

dition of hyperaemia should be the probable one is difficult to understand. 
The condition was evidently one of exhaustion, and the exhaustion of the 
nervous elements may well occur without the coexistence of a hyperaemic 
condition. The second case cited was evidently one of incipient meningitis, 
and if a hyperaemic condition was present it was simply the introductory 
stage of the graver disease. The third case of West is one in which we 
should be inclined at the present day to make the diagnosis of slight hem- 
orrhagic meningitis due to traumatism. 

In view of Schmauss's experience regarding the effects of concussion upon 
the spinal cord of animals we can readily understand why a hemorrhagic 
meningitis with a general hyperaemic condition of the meninges should fol- 
low upon concussion and direct injuries to the cord. But even granting this, 
we are not saying much in favor of establishing the condition of hyperaemia 
of the spinal cord as a distinct form of disease. 

In concluding this rather vague subject the most that can be said is that 
the less frequently one makes the diagnosis of spinal hyperaemia and the less 
value one attaches to the symptoms, so fully described by Hammond, the 
better it will be ; for such symptoms as the girdle sensation, muscular spasms, 
paralysis of both extremities, incontinence of the bladder and others, are in- 
cluded by Hammond under the symptomatology of spinal hyperaemia, and 
are unquestionably due to graver organic changes of the spinal cord. 

Treatment. — If there is reason to suspect an acute 
hyperaemia of the cord, the application of an ice-bag or of a 
number of leeches to the spine, is all that can be consistently 
done. Laxatives, small doses of the bromides and of chloral 
may be given to support the other measures. 



BIBLIOGRAPHY. 

Eisenlohr : Deutsche Med. Wochenschr., 1892, p. 49. 

Hammond : Diseases of the Nervous System, 8th edition, 1886, p. 391. 

Lichtheim : Verhandlungen des Congresses f. Innere Medicin, 1887, p. 84. 

Minnich : Zeitschrift f. klin. Medicin, 1892, vol. xxi., p. 25 and p. 264. 

Monti : Hyperamie, etc., Article in Gerhardt's Manual, p. 362. 

v. Noorden : Charite Annalen, 1891 and 1892. 

Nonne : Archiv f. Psychiatrie, vol. xxv., 1893. 

Roesebeck: Inaugural Dissertation. Gottingen, 1894. 

Spitzka : Pepper's System of Medicine, vol. v., p. 801. 



CHAPTER XVI. 

INFANTILE SPINAL PARALYSIS — THE ESSENTIAL PARAL- 
YSIS OF CHILDREN— POLIOMYELITIS ANTERIOR ACUTA. 

Few diseases present as many interesting- problems as 
does infantile spinal paralysis. While the diagnosis of the 
disease is an easy one, there is considerable doubt as to its 
true etiology ; its pathology, which was supposed to be 
firmly established, is still under discussion, and the .treat- 
ment of the disease deserves a most careful consideration. 
A full history of infantile spinal paralysis would convey a 
very fair idea of many of the important advances made in 
neurological science within the last twenty years. In no 
disease can the improved methods of studying the morbid 
anatomy of the spinal cord be put to better use, and in few 
diseases can the finer problems of electrical changes, of the 
localization of spinal functions, be studied to greater ad- 
vantage than in this acute spinal palsy. 

The clinical features of acute anterior poliomyelitis were recognized many- 
years ago by the German orthopedic surgeon Heine, and his description of 
the disease still holds good, with few exceptions, to the present day. If we 
add to his description of the disease what has been discovered since his day, 
regarding the electrical reactions and the behavior of the reflexes, we shall 
have an entirely satisfactory account of all the symptoms of the disease. 
But since the days of Heine innumerable French, German, English, and 
American authors, among whom we may name Duchenne, Charcot, Marie, 
Erb, Seguin, Striimpell, Seeligmiiller, Siemerling, Sinkler, Mary P.-Jacobi, 
and a host of others, have contributed to our knowledge of this disease. 
More recent authors, whose elaborate researches have added greatly to the 
proper conception of the pathology of the disease, will be referred to later on 
in connection with this special subdivision of the subject. 

Symptoms. — Acute anterior poliomyelitis comes on, in 
the vast majority of the cases, in the fashion of an acute in- 
fectious disease. Its onset is signalized by fever, vomiting, 
19 



290 



THE NERVOUS DISEASES OF CHILDREN. 



convulsions, and even coma. All these symptoms may last 
for a few hours, or even a few days, after which time they 
gradually subside and give way to the paralysis, which is 

the most permanent 
feature of the disease. 
The paralysis is typi- 
cal of all the palsies 
which are due to a le- 
sion in the second di- 
vision of the motor 
tract.* This is equiv- 
alent to saying that 
the paralysis is of the 
flaccid order; that it 
is associated with at- 
rophy ; that the elec- 
trical reactions in the 
paralyzed parts are al- 
tered, and that the re- 
flexes in the parts af- 
fected are diminished 
or lost. The very sud- 
denness of the onset is 
extremely character- 
istic of the disease. 
There are, as a rule, 
no prodromata, and 
even in cases in which 
such prodromal symp- 
toms have been re- 
ported, it is doubtful 
whether their occur- 
rence was not a mere 
coincidence. The fe- 
ver varies between 
102° and 104 F., rarely exceeding the latter for any consid- 
erable period of time. The fever generally lasts for twenty- 
four or forty-eight hours, though in some cases in which 

* This includes the ganglion cell in the anterior horns, the anterior roots, the pe- 
ripheral nerve, and the muscle. 




Fig. 74. — Case of Infantile Spinal Palsy; Paralysis 
and Atrophy of Left Leg chiefly. 



INFANTILE SPINAL PARALYSIS. 



291 



all the symptoms show marked severity the fever may last 
for an entire week. There is no doubt, however, that in a 
few cases of typical spinal infantile paralysis no fever what- 
ever occurs. Seguin was inclined to doubt this point, but 
the general consensus of opinion is in favor of the occur- 
rence of such an apyretic con- 
dition. The vomiting which ac- 
companies the fever, and sets 
in at a very early period of the 
disease, may resemble the cere- 
bral type, and is independent of 
any gastric disturbance. The 
convulsions are quite common 
during the first day of the dis- 
ease, are occasionally repeated 
during the first three days, some- 
times during the first week. 
They are of the order of general 
convulsions, and in this respect 
can be distinguished from the 
convulsions which occur during 
the earlier stage of acute cere- 
bral diseases. In some cases of 
poliomyelitis anterior acuta the 
convulsions are entirely absent. 
Coma is on the whole very much 
rarer than convulsions, and, if 
present, may be taken to indi- 
cate a tolerably severe form of 
the disease. The coma is not as 
profound, and not of as long duration as in many acute cer 
ebral diseases. 




Fig. 75.— Case of Acute Infantile 
Cerebral Palsy for Comparison 
with Fig. 74. 



I have drawn this analogy between the acute cerebral and the acute 
spinal disease, because as a matter of fact a sharp differentiation between 
the two is often impossible in the earlier days of the disease ; and many a 
case of incipient acute anterior poliomyelitis has been diagnosticated by 
skilful physicians as the first stage of a meningitis, of cerebral hemorrhage, 
and what not. 

If these symptoms of the initial stage are not well de- 
veloped it stands to reason that but few physicians would 



292 THE NERVOUS DISEASES OF CHILDREN. 

be willing to make the diagnosis of a spinal infantile palsy 
with absolute certainty. The diagnosis becomes positive 
only after the recognition of the form of palsy present. 

The paralysis may possibly be present from the very 
start, but it is very often overlooked in the presence of the 
other symptoms which appear to be so much more serious. 
I have in three different cases recognized the paralysis dur- 
ing the first ten hours after the onset of the first symptoms. 
The paralysis is widely distributed at the start. It may in- 
volve all the four extremities, and may even involve parts 
supplied by the lower cranial nerves (bulbar paralysis) as 
observed by Medin. In some few cases disturbances of 
speech (dysarthria rather than aphasia) have been noticed. 
But the initial wide-spread paralysis rapidly diminishes 
within a few days, or within the first week, and before long 
the physician will be able to recognize the parts which 
will remain permanently paralyzed, or at least to recognize 
those parts which will remain permanently more affected 
than others ; for it is not until after the lapse of some con- 
siderable time, say from two to four weeks, that the retro- 
gression of the paralysis ceases and an inference can be 
drawn as to the parts that will be permanently maimed. 
This retrogression of the paralysis has been insisted on by 
many authors as the most important feature of the early 
stages of the disease, but it should be added that in some 
instances the paralysis is seen to increase for a few days 
after the initial stormy symptoms have passed, then 
reaches its climax, and from this time on begins to recede 
until the few groups of muscles that are more seriously dis- 
eased give an idea of the amount of permanent injury done. 

The permanent paralysis may affect one or more parts of the body, but 
the lower extremities are much more frequently affected than the upper. In 
her article in Pepper's " System of Medicine," Mary Putnam-Jacobi, quotings 
from Duchenne and Seeligmiiller, shows that the paralysis was most fre- 
quently distributed as follows : Left lower extremity in 34 cases ; right lower 
extremity, 40 times ; right upper extremity and left upper extremity, 23. 
times ; all four extremities, 7 times ; both upper extremities, 3 times ; both 
lower extremities, 23 times ; left upper and lower extremity, twice ; right up- 
per and lower extremity, once ; right upper and left lower extremity, 3 times ; 
muscles of trunk and abdomen, once. Of these 137 cases it will be seen that 
the vast majority involve the lower extremities. The paraplegic and mono- 



INFANTILE SPINAL PARALYSIS. 



293 



plegic form are by far the most frequent, while among monoplegias an affec- 
tion of the lower extremity is more frequent than that of the upper ; paralysis 
of an upper extremity is, however, not so rare but that it is seen often 
enough in every clinic. Special mention should be made of a paralysis of 
the deep muscles of the back (which are involved in a number of the cases) 
which gives rise to extreme 
lateral curvature of the spine. 
The hemiplegic variety is very 
rare indeed. 

The further study of the 
exact distribution of the pa- 
ralysis in poliomyelitis has 
brought out a number of in- 
teresting facts. E. Remak in 
particular has shown that the 
parts paralyzed were function- 
ally, not anatomically, related. 
In the upper extremity the im- 
munity of the supinator longus 
in spite of paralysis of all of the 
extensor muscles in the fore- 
arm brings out this peculiarity 
as well as its affection in con- 
nection with paralysis of the 
deltoid, the biceps, and the 
brachialis anticus. This latter 
form of paralysis corresponds 
with the upper-arm type. In 
the lower extremity the pero- 
neal group of muscles is more 
frequently affected than any 
other; next in frequency the 
posterior tibial ; then the an- 
terior thigh muscles, and least 
frequently of all, the posterior 
thigh muscles. The tibialis 
anticus is generally paralyzed 
in connection with the quad- 
riceps extensor. These muscles are supplied by different nerves, but are 
associated in the extension movement of the leg during walking. The in- 
volvement of associated muscles in poliomyelitis would furnish the data for 
the study of spinal localization if the disease led more frequently to a fatal 
issue and to post-mortem examinations. 

An entire extremity, or a large group of muscles, may be permanently 
paralyzed ; in some cases the loss of function may be restricted to a single 
muscle. There is no little difficulty at times in making out the one or more 
muscles which have been permanently affected, and in a number of cases 




Fig. 76. — Paralysis of Upper-arm, with Atrophy 
(Left Side) due to an Attack of Poliomyelitis 
in Early Childhood. 



294 THE NERVOUS DISEASES OF CHILDREN. 

which have come under my observation a difference in the electrical behavior 
has been the only safe way of determining which muscle showed a departure 
from the normal. 



In addition to this retrogressive form of paralysis, which 
may at times be widely distributed and at other times curi- 
ously limited, we have to notice the rapidly developing 
atrophy of the paralyzed muscles. I have seen a marked 
difference between a paralyzed leg and the other normal 
member within three days of the first onset of the disease, 
and it is not at all unusual to recognize the wasting of the 
limb by superficial inspection within the first Aveek or two. 
The wasting is developed entirely in keeping with the dis- 
tribution of the paralysis, and this is so true that in one in- 
stance, which I can recall, the atrophy as well as the paral- 
ysis was evidently restricted to the upper portion of the 
tibialis anticus. In the case of the deltoid and the trapezius 
the clavicular portions only may be affected. The majority 
of the paralyzed limbs present a generally slender appear- 
ance. In some instances the difference between the two 
limbs may not amount to more than an inch or two in cir- 
cumference, in others the difference may be extreme, and it 
is not unusual to find a paralyzed extremity that is scarcely 
half the size of the normal one. The wasted paralyzed part 
makes the impression of an entirely limp, useless append- 
age, that is at the mercy of the parts whose muscles are 
still in a tolerable state of preservation. In addition to the 
reduction of the muscles the subcutaneous tissue and the 
fat often disappear, whereas in a few cases of children that 
were previously well nourished, the muscular wasting may, 
for a time, be concealed by the very considerable presence 
of adipose tissue. In addition to the reduction in circum- 
ference the atrophied parts do not grow as the normal parts 
do, whence it follows that short limbs are not infrequently 
the result of an early attack of poliomyelitis. 

The diseased member presents a few other symptoms 
which render the paralysis easy of detection. The skin 
over the paralyzed muscles often has a slightly shrivelled 
appearance ; more often still it is blue, cold, and clammy, so 
that by the mere touch of the two lower extremities the 



INFANTILE SPINAL PARALYSIS. 295 

physician can recognize the one that is paralyzed. This 
difference in the temperature of the parts is probably due to 
the improper blood-supply and to the natural shrinkage of 
blood-vessels that have no duties to perform, or else send a 
lessened supply to diminutive muscles. 

The behavior of the electrical reactions is of the utmost 
importance. With rare exceptions the paralyzed muscles 
and the nerves supplying them exhibit a complete reaction 
of degeneration. Both the nerves and the muscles fail to 
respond to the faradic current ; the nerves cannot be ex- 
cited by ordinary galvanic stimulation, and the muscle re- 
sponds in sluggish fashion, and often with an altered gal- 
vanic formula, the anodal closure contraction being equal 
to, or greater than, the cathodal closure contraction (see 
pp. 44 and 45). These changes in electrical behavior come 
on very early after the onset of the disease. From several 
examinations which I have been able to make in very early 
stages of the disease, I can assert that both the faradic and 
the galvanic response of the parts paralyzed are increased 
for the first two days, but then become rapidly diminished ; 
the nerves and muscles soon fail to respond to the faradic 
current, while the galvanic response may remain increased 
for a very long period of time ; this grows more sluggish, 
and finally there is no contraction except to very strong 
currents. It can be stated with some degree of certainty 
that those parts which continue to respond well to faradism, 
say after the lapse of a week or more, will not remain per- 
manently paralyzed, whereas the utter loss of function for a 
varying period of time of those muscles which at once 
fail to respond to the faradic current may be safely pre- 
dicted. During the later stages of the disease the return 
of the faradic response in any muscle, or a normal behavior 
during galvanic stimulation, Avill lead us to infer that the 
muscle, or muscles, in question mav recover their previous 
function, at least to a limited extent ; but muscles which 
exhibit marked electrical changes for a considerable period 
of time have suffered a very serious injury. 

The reflexes are diminished in cases of poliomyelitis 
anterior; but this is true only of those reflexes which are 
associated with the normal function of the paralyzed [nuts. 



296 THE NERVOUS DISEASES OF CHILDREN. 

Thus in a large proportion of the cases of poliomyelitis 
anterior the knee-jerk is absent ; but it is needless to say 
that it is not absent in cases of the cervical type, nor even 
in those cases in which only the posterior tibial muscles or 
the muscles of the feet are involved. 

I have in a number of instances been confronted by other physicians with 
an expression of grave doubt as to the diagnosis of poliomyelitis because of 
the presence of the knee-jerk, when a careful examination of the parts paralyzed 
proved that the paralysis was limited to the extensor muscles below the knee 
and to the posterior groups of muscles, while the quadriceps extensor and all 
the anterior thigh muscles were entirely normal. The mere presence of the 
knee-jerk under such conditions need not, therefore, militate against the cor- 
rectness of the diagnosis. 

If all the muscles of a given part are equally paralyzed 
and equally atrophied no contractures will set in, but all the 
parts will be equally limp. Thus the leg can be pushed to 
and fro by the slightest touch (Punchinello leg). Since 
locomotion and station are the results of an accurate bal- 
ancing between the extensor and flexor groups of muscles, 
it is but natural to expect that if one set of muscles is para- 
lyzed, the opposing muscles, being tolerably normal and 
having less work to do, will overact, and permanent over- 
action is expressed by contracture. As a rule these con- 
tractures do not appear until an attempt is made to use the 
affected limbs, but in a few instances — and some such have 
come under my notice — the contractures form while the 
child lies helpless on its back without any attempt what- 
ever at walking or at using the limb. It would seem from 
this that some more active cause must be at work, and it is 
probable that the inflammation which destroys the function 
of some cells may cause an irritation of neighboring cells 
and fibres, and thus produce contracture, very much as it 
it is produced in other diseases in which a lesion in the ad- 
jacent white matter acts as an irritant upon the ganglion 
cells of the anterior horns. According to Volkmann and 
others the mere weight of the body or of a limb may cause 
a deformity if the muscles surrounding a joint are paralyzed. 

The most frequent deformities are as follows : Pes equinus, or equino- 
varus ; pes valgus ; genu recurvatum and incurvatum ; permanent flexion 
(rare) ; all these deformities are evidently promoted by the attempt to use the 



INFANTILE SPINAL PARALYSIS. 297 

maimed legs. Deformity of the hip is very much rarer than those affecting 
the knee or the foot. If the disease involve the upper extremities extension 
of the wrist is common in the presence of paralysis of the flexors ; clawed 
hand if the interossei are involved, and a flexion of the fingers or wrist if the 
extensors chiefly are paralyzed. • The elbow-joint is rarely deformed, but is 
often entirely useless if the upper-arm muscles are paralyzed. At the shoul- 
der-joint atrophy of the deltoid may be the direct cause of a subluxation. 
The most serious deformities that occur are those due to a paralysis of the 
abdominal and deep spinal muscles. According to the extent and number of 
the muscles involved we may have a scoliosis, occasionally a kyphosis ; but 
most frequently very marked lateral curvature, particularly in the cervical re- 
gion, or a very marked lordosis in the lumbar region. From paralysis of the 
abdominal muscles the abdomen may become peculiarly distorted, and in one 
case of a child of about three, which I saw in private practice, a marked lordosis 
of the lumbar region was associated with what appeared to be an enormous 
bulging of the anterior left half of the abdomen. In accounting for these de- 
formities we must remember that in addition to the paralysis of the muscles, 
the arrested growth of the bones, and the disturbance in the general develop- 
ment of the child play a very considerable part. 

Before leaving the symptomatology of the disease it is 
important to insist on a few negative symptoms which help 
to corroborate the diagnosis in doubtful cases. It is gener- 
ally asserted that pain is entirely absent. This is true 
of the vast majority of cases, and particularly of the later 
stages of the disease ; but in one of the few cases which I 
was fortunate enough to see at an early day, pain was an ex- 
tremely prominent symptom. In such cases, therefore, the 
absence or presence of pain could not serve as a sufficient 
factor in differentiating the disease from a peripheral neu- 
ritis, or from articular rheumatism. The bladder and the 
rectum are, as a rule, not involved. This is a point of some 
importance in helping to differentiate between inflammation 
of the anterior horns and a general myelitis — a point of dif- 
ferential diagnosis that comes up frequently enough for dis- 
cussion. The entire absence of cerebral symptoms after 
those of the initial stage have passed away will help to dis- 
tinguish the spinal infantile paralysis from many cases of 
cerebral palsy. 

Summarizing all these symptoms we may state that the 
diagnosis of poliomyelitis anterior may be made if the 
paralysis, however widely distributed, or however narrowly 
limited, and in whatever part of the body, comes on after 



298 THE NERVOUS DISEASES OF CHILDREN. 

an acute onset marked by fever, vomiting, and convulsions, 
and if this paralysis is associated at an early da)' with 
atrophy, with changes in electrical reactions, and with a loss 
of reflex activity in the paralyzed parts. 

Morbid Anatomy and Pathology. — " Poliomyelitis 
anterior due to atrophy of the ganglion cells of the anterior 
horns," has become a byword of neurological science. It 
would be fortunate indeed if such a simple statement as this 
would settle the much-disputed question of the morbid 
changes underlying the disease. 

As far back as 1863, Cornil published a case of a woman who had ac- 
quired a sudden paralysis of both legs at the age of two years, and who had 
died of carcinoma at the age of forty-nine. Cornil found the spinal cord 
much smaller than normal, and thought this due to an atrophy of the anterior 
roots and of the antero- lateral white matter. He referred to atrophic gan- 
glion cells, but did not make them responsible for the disease. In 1865 Pre- 
vost and Vulpian examined another case, also in an elderly person, who had 
died of cerebrospinal meningitis. She had had a paralytic club-foot with 
complete atrophy of the posterior leg muscles, which was found to be due to 
an atrophy of the anterior horns of the left side, in the lumbar enlargement. 
The ganglion cells were found to be fewer in number, and the antero-lateral 
white matter was also atrophied on the same side. Very much the same 
result was obtained in another case by Clarke, but the importance of disease 
of the gray matter and of the relations of the ganglion cells of the anterior 
horns to the paralyzed parts was not fully established until the famous publi- 
cation of Charcot and Joffroy in 1870, who made the atrophy of the ganglion 
cells entirely responsible for all the symptoms of the disease. Their studies 
were based also upon the examination of the spinal cord of a woman, who 
died at the age of forty, but who had acquired an infantile paralysis at the 
age of seven years. The ganglion cells in the lumbar segment were dimin- 
ished, and were in part entirely absent. A dense sclerotic tissue marked the 
disappearance of these ganglion cells. 

Since the publication of Charcot and Joffroy's article, it 
was current belief that the chief anatomical changes in polio- 
myelitis were those involving the gray matter and its gan- 
glion cells; that the morbid process might extend through- 
out the entire length of the cord, or it might be developed 
chiefly in the cervical or lumbar enlargement. The question 
arose whether the inflammation of the anterior horns is pa- 
renchymatous in character, that is, a primary inflammation of 
the ganglion cells, or whether it is of the order of interstitial 
inflammations, in which the neuroglia is the part first affected 



INFANTILE SPINAL PARALYSIS. 2 99 

and the ganglion cells are destroyed later on. This dispute 
has been carried on to the present day. It was begun by 
Roger and Damaschino, and has been continued by Leyden, 
Schultze, Eisenlohr, Bramwell, and a number of others. 
Leyden recognized that a number of different pathological 
processes might give rise to the clinical symptoms of an 
infantile spinal palsy. He was inclined to adopt Charcot's 
theory of a parenchymatous inflammation, but in one case 
which he examined he thought the lesions somewhat similar 
to those described by Roger and Damaschino. It was evi- 
dent that this question could not be satisfactorily settled if 
the microscopical investigations were restricted to cases in 
which many years had passed between the time of onset of 
the disease and the death of the patient. 

The effort has been made to study recent cases of the dis- 
ease. The first good opportunity was presented by a very 
extensive epidemic of poliomyelitis anterior acuta observed 
by Professor Medin, of Stockholm, and carefully. described 
by his pupil John Rissler, Avho recorded the autopsies on 
three cases. Goldscheider, Dauber, and Siemerling have 
also published the results of post-mortem examinations 
of children who died very shortly after the onset of the 
disease. These German authors have helped greatly to de- 
velop a fuller knowledge of the anatomical process, and 
their results are in direct contradiction of the opinions of 
Charcot and Joffroy, which have obtained such general cre- 
dence in neurology. Nearly all recent writers * have come to 
the conclusion that the entire gray matter is the seat of in- 
terstitial inflammation, and that the changes in the ganglion 
cells are secondary. Many of the authors are also inclined 
to regard poliomyelitis as an acute infectious disease, in 
spite of the entire absence, up to the present day, of proof of 
the microbic origin of the disease. They are of the opinion 
that the entire gray substance is easily affected and infected 
by the poison, and that that part of the spinal cord is most 
easily involved which has the most abundant blood-supply 
(see Fig. 73). 

Goldscheider's investigations, which were most carefully 
made, point to the important role played by the blood-ves- 

* Rissler and v. Kahlden still hold to Charcot's views. 



3<x> 



THE NERVOUS DISEASES OE CHILDREN. 



sels of the spinal cord. The author concludes that a condi- 
tion of irritation is present in the walls of the blood-vessels 
which leads to a dilatation of these vessels and to a prolif- 
eration of their endothelial elements. From this the morbid 
process extends to the neuroglia and produces a prolifera- 
tion of its cells. The changes in the ganglion cells are of a 




Fig. tj. — Poliomyelitis Anterior ; Part of an Acute Myelitis. Death of child (age, two 
and a half years) eight days after onset of complete palsy of legs and arms. (Sie- 
merling.) Section through lumbar segment, showing disruption of anterior gray 
matter from hemorrhage into it. B, marginal blood-vessel ; A, branch of anterior 
spinal artery. 



degenerative nature, and the changes in them, as well as in 
the nerve-fibres, are secondary and due to disease of the 
blood-vessels. Goldscheider has also shown that these de- 
generative changes occur most distinctly in the vicinity of 



INFANTILE SPINAL PARALYSIS. 30E 

altered blood-vessels, and that the degenerated ganglion 
cells lie in vascular areas. 

The cases reported by Siemerling, while corroborating 
the views of Goldscheider and others, have a still deeper 
significance. They show that in some instances a poliomy- 
elitis anterior is merely a part of a general myelitis of both 
the gray and the white matter. The frequent restriction 
of the process to the anterior cornua must be ascribed alto- 
gether to the peculiarities of arterial blood supply. It is 







Fig. 78. — Poliomyelitis Anterior of Old Standing, showing Disappearance of Ganglion 
Cells on Right Side, and Shrinkage of Right Half of Cord. Drawn from a section 
through lower cervical region. 

doubtful, however, whether the hemorrhage into the an- 
terior horns is often as destructive as in one of Siemerling's 
cases (Fig. yj). All these investigations prove that in in- 
fantile spinal palsy the inflammatory process is interstitial 
not parenchymatous. It may be limited to a few segments 
of the cord, or it may involve the greater part of the cord, 
and may extend to the medulla and pons. 

For the changes that occur in consequence of this early 
inflammation, and for the appearance of the cord in the later 






302 



THE NERVOUS DISEASES OF CHILDREN. 



years, we may refer safely enough to the famous publica- 
tions of Charcot and others. 

There is, first of all, a disappearance, often complete, of 
the larger ganglion cells of the anterior horns, and the few 
that are visible are altered in appearance. The nucleus has 
disappeared, the cell-body is shrunken, and the cell con- 
tours are entirely different from the normal, every trace of 
the cell processes having disappeared. But the ganglion 
cells are not the only parts that undergo changes ; the en- 
tire gray matter shrinks, and, as can be seen in the annexed 
cut, the entire gray matter in one-half of the spinal cord is 

shrunken in size, and 
the white matter of the 
same side is also very 
much less in volume 
than that of the opposite 
half. In consequence of 
the changes going on 
in the gray matter, the 
columns of Clarke dis- 
appear, together with 
other nervous struct- 
ures. These changes 
are unquestionably sec- 
ondary to the changes 
in the anterior horns, 
and considering the intimate relation between the anterior 
horns and the remaining part of the gray matter, as has 
been brought to light by recent anatomical investigations, 
we can readily understand why, in consequence of disease 
in one part of the gray matter causing destruction of nerve- 
cells, the nerve-fibres which owe their life and nutrition to 
such cells disappear as well. The anterior nerve-roots are 
smaller than the corresponding roots of the sound side. 
These changes are also, in all probability, secondary to the 
changes in the ganglion cells. 

So much for the changes to be observed in the spinal 
cord itself. The atrophied muscles also present character- 
istic conditions. The fibres are very much diminished in size, 
many of them have disappeared altogether, and the place 




Fig. 79. — Poliomyelitis Anterior. Chronic stage ; 
section through sixth cervical segment, showing 
diminution of anterior gray matter and of en- 
tire half of right side. (Drawn from a specimen 
kindly furnished me by Dr. Collins. ) 



INFANTILE SPINAL PARALYSIS. 303 

once occupied by the normal fibres is largely filled by adi- 
pose tissue. There is in these cases no such nuclear prolif- 
eration and no hypertrophy of fibres such as are found in 
the muscles of patients suffering from various forms of 
muscular dystrophy. But even the presence of a few 
hypertrophied fibres would not be unusual, as the stage of 
hypertrophy seems to indicate an incipient irritation which 
precedes the condition of atrophy. Marie has gone to some 
trouble to show that even the bones in cases of poliomye- 
litis undergo trophic changes. The bones are smaller than 
those of the corresponding healthy member and appear 
more rounded on cross-section than the healthy bone does. 
Theory of the Disease. — That poliomyelitis repre- 
sents an acute inflammatory condition of the anterior gray 
matter of the spinal cord is conceded on all sides, but the 
question arises what the origin of such inflammation may 
be. The only satisfactory explanation at the present day is 
to suppose that the inflammation is the result of an acute 
infection which happens to be located in the spinal cord, 
just as other acute infectious diseases show a predilection 
for other sites in the body. The microbic origin has not 
yet been satisfactorily demonstrated, but all the clinical 
facts point toward this view, and the close dependence of 
the myelitic process upon the distribution of the blood-ves- 
sels lends further color to this theory. The infectious ori- 
gin of poliomyelitis is also rendered highly probable by the 
frequent observation of the epidemic occurrence of infantile 
spinal paralysis. Such epidemics have been recorded by 
Medin and Briegleb in Europe, and by Colmer (1843) anQ " 
Caverly (1894) in this country. 

For several years past I have recorded carefully the cases of poliomyelitis 
in dispensary and private practice, and have noticed that at least 75 per 
cent, began between the months of July and October. Medin observed rive 
cases in the spring of one year, and between August and November he had 
examined altogether forty-four cases of poliomyelitis which had begun during 
this period. Sinkler states that of 270 cases 213, or 78.8 per cent., were at- 
tacked in the hot months of the year, from May to September, inclusive. 

Marie supposes that an infectious embolism or thrombo- 
sis in one or more of the branches of the anterior spinal 



304 THE NERVOUS DISEASES OF CHILDREN, 

artery may be the direct cause of the attack of poliomye- 
litis. 

Marie is inclined also to infer the infectious nature of poliomyelitis from 
the close resemblance between polioencephalitis and poliomyelitis, and quotes 
approvingly the two cases of Moebius occurring in one family, in which one 
child was attacked with the form of acute cerebral palsy, and the other child 
with an acute spinal palsy. But surely this proof of the infectious theory of 
acute cerebral palsy is extremely slender, and Moebius's cases might well be 
due to a coincidence rather than to an infection, which is supposed to have 
caused a cerebral paralysis in the one, and a spinal paralysis in the other 
child. Nor can two cases occurring in the same family be considered evi- 
dence of an epidemic character of the disease. A few. years ago two chil- 
dren, cousins, were brought to me, who had developed acute spinal palsy 
within two weeks of one another. The one child has remained severely 
paralyzed in both lower extremities up to the present day, the other child 
escaped with a slight paralysis of the anterior tibial group. The theory of 
infection would be a very simple one to hold in such cases, but on closer 
examination it was found that both these children had developed the symp- 
toms of their disease shortly after exposure to an extremely cold surf bath. 
The infectious theory of poliomyelitis is a very plausible one, but we cannot 
disregard other possible causes, and refrigeration, as in the two cases just 
cited, may in some instances be a powerful factor in the development of 
poliomyelitis. It seems to me that those authors make a mistake who insist 
on a single origin for such a frequent disease as poliomyelitis. Many, pos- 
sibly most, cases may be due to an infectious cause, but some may be due 
to refrigeration, others to slight traumatic injuries. Marie is right in holding- 
that dentition is never the actual cause of poliomyelitis, but during the period 
of dentition other influences may bring about the disease at a time when 
the spinal cord, as well as the general nervous system, is in a condition of 
extreme irritability. What we have just said regarding the theory of the 
disease also includes all that can safely be stated regarding the etiology. 

Differential Diagnosis. — Poliomyelitis is most fre- 
quently confounded, during its acute stage, with acute cere- 
bral conditions, such as meningitis or some form of acute 
cerebral palsy. Meningitis can be safely excluded if there 
are no other signs of a meningeal process, except possibly 
coma and convulsions. In cases of meningitis these consti- 
tute the first of a series of many cerebral symptoms, such 
as vomiting, rigidity of the neck, headaches, cranial nerve 
affections, and the like. In poliomyelitis none of these 
symptoms appear, and the coma and convulsions last but a 
relatively short time. 

There is little difficulty in distinguishing between well- 



INFANTILE SPINAL PARALYSIS. 305 

developed cases of acute spinal and acute cerebral palsy of 
children, but the less pronounced types of these diseases 
cannot be easily distinguished from one another unless a 
very careful examination is, made of all the accompanying 
symptoms. The mode of onset may be exactly similar in 
both ; it is, in fact, on the close resemblance between the 
two diseases in this respect that Striimpell was led to 
build up his theory of the analogy between the two. But 
aside from the symptoms of onset, the clinical features are 
almost diametrically opposed to one another. The follow- 
ing table will bring out these symptoms in the clearest pos- 
sible manner: 

Acute Spinal Palsy. Acute Cerebral Palsy. 

Onset sudden, with fever, coma, and Onset sudden, with fever, coma, and 

convulsions. Convulsions rarely convulsions. Convulsions apt to be 

repeated after first few days. repeated. 

Paralysis flaccid, associated with Paralysis spastic ; no atrophy ; asso- 

atrophy. ciated with rigidity and contrac- 
tures. 

Paralysis widely distributed, possibly Paralysis generally hemiplegic, some- 
involving all extremities, or nar- times diplegic or paraplegic. Mono- 
rowly limited to one member, or plegia rare, 
even a single group of muscles. 

Electrical reactions altered (R. D.). Electrical reaction normal. 

Deep reflexes diminished or lost. Deep reflexes exaggerated. 

Intellect never permanently involved ; Intellect often involved ; epilepsy fre- 

no epilepsy. quent. 

Doubt may arise as to the differential diagnosis in some 
cases between poliomyelitis anterior and a peripheral (mul- 
tiple or simple) neuritis. The onset may be equally sudden 
in both, though in many cases of neuritis the onset is much 
more gradual than it is in cases of poliomyelitis. In neu- 
ritis there are, as a rule, fewer symptoms of general ner- 
vous disturbance than in poliomyelitis ; but in those forms 
of neuritis in which there is a distinct toxic infection the 
toxic poisoning may produce cerebral symptoms very 
closely akin to those met with in the earlier stages of polio- 
myelitis. The distribution of the paralysis may be the 
same in both instances, but after all it is much more likely 
to be distributed according to strict anatomical lines in 



306 THE NERVOUS DISEASES OF CHILDREN. 

neuritis than in poliomyelitis. In the latter, muscles that 
have a common function are very apt to be paralyzed to- 
g-ether. In former days the presence of pain along nerve- 
trunks and along nerve-branches supplying the paralyzed 
muscles was supposed to be a safe feature of differential 
diagnosis, and this holds good in a majority of cases ; but 
according to my own experience pain may be present in the 
acute stage of poliomyelitis, and if the child is too young 
to give accurate information to the physician, it is well- 
nigh impossible to determine whether the pain is a gen- 
eral one in the joints or whether it is along the distribution 
of the peripheral nerve-branches. But 1 have never seen 
pain persist for any great length of time in poliomyelitis, 
while it persists, as a rule, for days and weeks in cases of 
neuritis. The atrophy, the electrical reactions, and the re- 
flexes may be as thoroughly affected in one disease as in 
the other. The differential diagnosis can, in many in- 
stances, be made only after a close observation of the en- 
tire course the disease has taken. (See Chapter on Multi- 
ple Neuritis.) 

The various forms of progressive muscular dystrophies 
may occasionally be mistaken for poliomyelitis and vice 
versa. In cases that are seen years after the onset of 
the trouble, the initial history of the case will often be 
an important guide to diagnosis. If a case of typical mus- 
cular dystrophy is seen during the stage of atrophy, a su- 
perficial inspection of the case may suggest an old polio- 
myelitis ; but in the progressive dystrophies the atrophy 
affects an entire limb rather than single groups, or if it has 
spread to several extremities it is, as a rule, much more 
general than in cases of poliomyelitis, while in the latter 
the electrical changes are, as a rule, much more complete 
than in cases of progressive dystrophies. 

One special form of progressive muscular atrophy is easily confounded 
with the chronic stages of acute or subacute poliomyelitis; I refer to the 
peroneal form of progressive muscular atrophy, the Charcot-Marie type, 
cases of which have been recorded by Tooth, Hoffmann, myself, and others. 
This atrophy, beginning in the peroneal group of muscles, may for a time 
simulate a poliomyelitis involving this same group ; but the upward march 
of the disease in the case of progressive muscular atrophy, the progression 



INFANTILE SPINAL PARALYSIS. 2>°7 

rather than retrogression, the involvement of the upper extremities, the strictly 
bilateral character of the atrophy and the slow development, the incomplete 
changes in electrical reactions, will help to distinguish the peroneal form of 
progressive muscular atrophy from poliomyelitis ; but the differential diag- 
nosis requires the most careful examination, and in some instances the hered- 
itary or family taint, in cases of progressive muscular atrophy, will give the 
correct clew to the nature of the disease. 

Prognosis. — Altogether too gloomy a prognosis is gen- 
erally given in poliomyelitis. This is based upon the 
fact that some palsy always remains, but the actual res- 
idue of palsy may be so slight that one should be care- 
ful not to depress the hopes of parents and patient. Above 
all, there is no need of predicting that the child will re- 
main a hopeless cripple for life. There is no telling at the 
outset of the disease to what extent the retrogression may 
take place ; but, of course, the more widely distributed the 
paralysis is at the beginning the larger the remaining palsy 
is apt to be, although some cases which begin in very 
stormy fashion exhibit more progress than those which be- 
gin less violently. Cases in which but a few muscles are 
paralyzed at the start often recover with very little per. 
manent injury. Very little change need be expected in 
the first few weeks of the disease, but there is reason to 
hope that those parts which show any improvement within 
the first few weeks or months after the onset of the disease 
will recover power before long, and only those parts will 
remain permanently paralyzed which after months show no 
signs of improvement. Muscles which are paralyzed, but 
which exhibit slight, or no changes of electrical reaction, 
may be regarded in a hopeful light, and, on the other hand, 
those which very soon after the onset of the palsy exhibit 
distinct reaction of defeneration, and for months afterward 
show no sign of change in this respect, are apt to be per- 
manently paralyzed. The more complete the wasting of 
the muscles, the less likely these muscles are to recover ; 
and if contractures form in the opposing groups permanent 
disability is the probable result, but even such disability 
can often be remedied by surgical procedures. 

The prognosis as regards life is, with few exceptions, en- 
tirely favorable. Cases that end fatally are apt to do sc-> 



308 THE NERVOUS DISEASES OF CHILDREN. 

within the first few weeks of the disease. But parents are 
often most grateful for the assurance that if the child sur- 
vives, however great the paralysis may be, its mental de- 
velopment will in nowise be impaired. This assurance can 
only be given if the physician is certain that the palsy is due 
to a spinal lesion and is not a form of cerebral paralysis. 

Treatment. — During the acute stage of an anterior 
poliomyelitis the general condition only should be treated 
and little attention need be paid to the paralysis. The child 
should be kept in a quiet room, mild antipyretic measures 
may be employed, such as small doses of phenacetin, of anti- 
pyrin, of the salicylates, and the like. In the earlier stages 
cold applications, or mild counter-irritation over that part 
of the spine which is involved in the given case, are quite in 
order ; and the attempt should be made to limit the spread 
of the inflammation by the administration of small doses of 
bromide and of ergot (a few drops of the fluid extract). 
Iodides and other drugs I have found to be utterly ineffi- 
cient. The child should during this period be carefully fed, 
and the bowels should be thorougly purged by the use of 
small but sufficient doses of calomel. After the acute stage 
is passed the paralyzed muscles demand treatment. Elec- 
tricity and massage are the most effective therapeutic 
measures. Avoid electrization of the spine ; first, because 
it is not at all certain that the electric current reaches the 
spinal cord, and secondly, because the use of strong cur- 
rents makes the child extremely restless and may do more 
harm than good. In the treatment of paralyzed muscles an 
important use of the electric current is to exercise muscles 
which are no longer subject to the will ; it supplies, in 
other words, a convenient form of gymnastics. The cur- 
rent may, in addition, improve the state of the paralyzed 
and atrophied muscles, but whether or not it increases the 
conductive powers of paralyzed nerve and muscle I am not 
willing to assert. Since we wish to make the muscles con- 
tract, the only form of current that is serviceable is that 
form to which the muscle will respond. If the reaction of 
degeneration is complete the faradic current is quite useless, 
and if the diagnostic tests have shown that the muscles re- 
spond to the anode better than to the cathode, exercise by 



INFANTILE SPINAL PARALYSIS. 3 00. 

anodal opening or closing of the current is the only proper 
method. 

This should be done in sittings of ten to fifteen minutes once or twice a 
day, and that strength of current should be employed which is sufficient to 
produce mild contractions. Excessive contractions are not called for and 
help to increase the difficulties of application. If a muscle responds to the 
faradic current, however slightly, that current should be employed together 
with the galvanic, and it has been my habit, even in the more severely par- 
alyzed cases of poliomyelitis, to make occasional tests with the faradic cur- 
rent, first, in order to determine whether there is any sign of improvement in 
any group of muscles that have been paralyzed, and, secondly, to give such 
muscles the benefit of both currents. If the muscles react at all to both cur- 
rents, both may be employed in one and the same sitting, or given in alter- 
nate sittings. I am a thorough believer in the good influence that massage 
has upon atrophied and palsied muscles. It helps undoubtedly to keep up 
the nutrition of such parts, and in cases in which there is an incipient ten- 
dency to contracture such tendency may be overcome by the proper use of 
massage. But this should, if possible, be intrusted to skilled manipulators 
and not to the mother of the child or to a nurse, whose " rubbings " are, as a 
rule, wholly ineffective. Passive movements are entirely in order, and some 
good results have been attained by the regular use of Swedish movements, 
such as are given by trained rubbers or by a regular system of treatment in a 
well-equipped Zander institute. Unfortunately such institutes are for the 
present found only in a few large cities. 



During the chronic stages of poliomyelitis orthopedic 
measures should be employed without reserve. If the con- 
tractures have persisted for years they will not disappear 
without treatment. Tenotomies are followed by results 
fully as favorable as those in chronic ocular palsies. The 
general condition of the child and the condition of the 
paralyzed muscles improves markedly after the orthopedic 
surgeon has done his work, and such tenotomies need not 
be restricted to the tendon Achillis, but many of the other 
muscles which are in a state of contracture can as well be 
similarly treated. The application of splints according to 
the best orthopedic principles is also of great assistance to 
the child, and this, too, should not be delayed too long, for it 
is far better to have a child walk in splints than to have 
ugly deformities of the joints develop which may cripple 
him for life. Since I have recognized the truth of these 
principles I have had the satisfaction of seeing children, 



310 THE NERVOUS DISEASES OF CHILDREN. 

and even very young children, walk within a few months 
after the development of a poliomyelitis, while in former 
years such children were compelled to be carried about in 
the arms of a nurse or to be wheeled about in chairs. I am 
satisfied that few cases of poliomyelitis are so severe that 
much cannot be clone by the proper application of ortho- 
pedic measures. In cases in which the joints are entirely use- 
less on account of the complete atrophy of the muscles, the 
operation for arthrodesis, as suggested by Wolff and others, 
may be resorted to. In this way a leg that would other- 
wise be entirely useless may be made to subserve the func- 
tion of standing and walking, though of course the station 
and the gait of the person will always be far from normal. 
At every stage of poliomyelitis active interference and 
active treatment are called for, and much harm and misery 
may be avoided if the physician, instead of quietly sitting by 
and saying that nothing can be done, will exercise his own 
mechanical ingenuity in every case to put the limb in the 
best possible condition for walking, whether such is to be 
attained by the use of splints or by one of the several or- 
thopedic measures that have been suggested. 

SUBACUTE ANTERIOR POLIOMYELITIS. 

Subacute anterior poliomyelitis is practically a mere variety of the acute 
form of the disease, and for that reason needs but little special mention. 
The entire difference between the two diseases is in the mode of onset, and ac- 
cording to our present views of the character and origin of acute spinal palsy, 
the subacute variety necessarily implies a milder form of infection than in the 
cases with a more acute and more violent beginning. The difference in the 
symptoms is also confined entirely to the difference in the manner of onset 
and the manner in which the paralysis is developed. In these cases of the 
subacute variety we find that the disease comes on very gradually. The 
child is ill at ease for some days or weeks, complains of weariness in walking, 
of pains in the joints and muscles. After some little while a decided paresis 
of one or more groups of muscles is observed, generally in the lower extremi- 
ties. This increases, and after a week or more a distinct paralysis is devel- 
oped. The paralysis then increases in the affected muscles, and is apt to 
spread somewhat after the fashion of a progressive muscular atrophy from 
one group of muscles to another. There is, therefore, a progression in these 
cases at the start, but the limit is very soon reached, and from this time on a 
retrogression again sets in, though not in such a marked degree as in the 
acute cases. The fact that the retrogression occurs after a given period of 



INFANTILE SPINAL PARALYSIS. 



311 



time will dispel all fears as regards the possibility of a progressive form of 
muscular atrophy. As a good illustration of this type, I may refer to a young 
girl who has been under my observation for many years. When the disease 
began she was thirteen years of age. A slight difficulty in the use of the 
toes was the first symptom the girl complained of, with the exception of a 
general weakness and an inability to walk long distances. From the exten- 
sors of the toes this spread to the tibialis anticus, and to the other muscles 
of the anterior tibial group. After a little the anterior thigh muscles, too, be- 
came involved, so that the girl was totally paralyzed for a period of several 
months. There was no history of fever at any time, or of any other symp- 
tom of an acute onset. The disease then came to a standstill ; the anterior 
thigh muscles recovered completely, and the paralysis is now, after the lapse 
of four years, entirely restricted to the tibialis anticus, and the extensor mus- 
cles of the toes. But even in the tibialis anticus the electrical reactions are 
quite normal, and the marked symptoms of reaction of degeneration are 
present only in the extensor muscles of the toes, which were those first 
affected. The knee-jerk, which was absent for many years, can now be 
elicited by Jendrassik's method, and the girl, who was once unable to use the 
limb at all, is now able to walk with but a slight halt in her gait. 

The differential diagnosis of such cases is often difficult, and cases are 
confounded easily with cases of progressive muscular atrophy, particularly of 
the peroneal form, and with cases of chronic neuritis ; but the points of dif- 
ferential diagnosis which were given between these diseases and the acute 
form of anterior poliomyelitis w r ill also help us in arriving at a correct diagno- 
sis of the subacute variety. 

Treatment. — The treatment should be conducted on the same princi- 
ples as were enunciated in the preceding discussion on acute spinal palsy. In 
those cases in which the original disease is not nearly so violent as in the 
acute form even more can be hoped from an early application of therapeutic 
measures, but it should be remembered that the natural course of the disease 
tends much more to recovery than does the acute form, so that tenotomies 
and other surgical procedures should be delayed until the disease has become 
entirely stationary, and there is no reason to think that further spontaneous 
recovery will take place. 

BIBLIOGRAPHY. 



Briegleb : Inaugural Dissertation. Jena, 1890. 
Caverly : New York Medical Record, 1894, vol. xlvi., p. 673. 
Charcot et Joffroy : Arch, de Physiologie, Paris, 1870, p. 134. 
Collins, J. : New York Medical Journal, January, 1894. 
Colmer, G. : American Journal of the Medical Sciences. 1843. 
Dauber : Zeitschrift f. Nervenheilkunde, vol. iv. 
Goldscheider : Ztschr. f. kl. Med., vol. xxiii., 1893, p. 494. 
Jacobi, Mary P.-: Article in Pepper's System. 

Kahlden, C. Von : In Ziegler's Beitrage, 1893, vol. xiii. Centralbl. fur Path. 
September 14, 1894. 



312 THE NERVOUS DISEASES OF CHILDREN. 

Leyden : Arch. f. Psychiatrie, vol. vi., p. 271. 

Marie, P. : Maladies de la Moelle. Paris, 1892. 

Medin : Proceedings of Tenth International Congress, vol. ii., div. vi. 

Roger etDamaschino : Comptes Rend, de la Soc. d. Biologie, 1871. 

Schultze : Neurolog. Centralbl, 1892, No. 19. Virchow's Arch., vol. lxviii. 

Seeligmiiller : Gerhardt's Handbuch, vol. v. 

Siemerling : Arch. f. Psychiatrie, vol. xxvi., p. 267. (Full literature to 1894.) 

Sinkler : Keating's Cyclopaedia, p. 683. 

Volkmann : Volkmann's Sammlung No. 1. 

Wolff, J.: Berl. kl. Wochenschr., 1886, No. 52. 



CHAPTER XVII. 

ACUTE MYELITIS. 

Myelitis, or inflammation of the spinal cord, has been 
made to cover a multitude of diagnostic sins, both in the 
adult and in the child. If we subdivide the cases accord- 
ing to the mode of onset, we may distinguish between an 
acute, a subacute, and a chronic form ; and if the classifica- 
tien is based on the origin of the myelitis we have an idio- 
pathic form, a traumatic myelitis, a tubercular, and a syphi- 
litic variety. As the symptoms are very much the same, 
whatever the original cause of the disease may be, it will 
be better to describe the characteristic features of acute 
myelitis, which occurs frequently enough in children to de- 
mand special study. 

Symptoms. — The symptoms in a given case will vary ac- 
cording to the site of the inflammation, and the intensity of 
the process. Its clinical features will depend upon the 
amount of cord tissue involved. In such cases more than 
in any others an accurate knowledge of the functions con- 
nected with each segment of the cord is of importance if a 
satisfactory diagnosis is to be made. In every case of com- 
plete transverse myelitis, at whatever level the area of in- 
flammation may be, motion and sensation are chiefly af- 
fected, the reflexes are disturbed, and the functions of the 
bladder and rectum are deranged. Bilateral paralysis (para- 
plegia) is the natural result of a myelitis. If the lesion is in 
the cervical portion of the cord, both upper and both lower 
extremities will be paralyzed ; the first because the very 
parts which are most intimately connected with the motion 
of the upper extremities are destroyed ; and the lower ex- 
tremities are involved because the fibres going to them are 
interrupted at the site of the lesion. The bilateral character 



3H 



THE NERVOUS DISEASES OE CHILDREN, 



of a palsy, is after all, the one symptom which points more 
frequently to a spinal lesion than any other. 

Almost the only exceptions to this rule are those cases in which a multi- 
ple neuritis, an ascending Landry's paralysis, or double cerebral lesions 
give rise to a bilateral form of palsy. 

If the lesion is in the cervical portion of the spinal cord, 
the paralysis of the upper extremities will be of a flaccid 
order; the paralysis of the lower extremi- 
ties will be spastic in character. Anaesthesia 
will be present in the four extremities and 
in the trunk to the level of the diseased seg- 
ments ; pupillary symptoms, Unilateral blush- 
ing (due to lesion of the sympathetic), and 
paralysis of the diaphragm are present in 
some cases. If the lesion is in the lumbar 
portion of the cord, the paralysis is re- 
stricted to the lower extremities and will 
be of a flaccid character, with more or less 
atrophy. If the lesion is in the cervical or 
dorsal portion of the cord, and the lumbar 
portion is entirely free from disease, the 
paraplegia of the lower extremities is of 
the spastic order, and the reason of this can 
be easily understood if we recall the fact 
that after a transverse lesion in any por- 
tion of the spinal cord the lateral columns 
will degenerate downward from that level 
(Fig. 80), and that such degeneration of the 
lateral columns in the presence of normal 
gray matter of the lumbar segments will 
produce a spastic form of paralysis with 
rigidities and contractures. Taking all 
cases of myelitis, the largest number affect the dorsal re- 
gion \ and in these the arms go free, as their spinal centres 
are above the site of the lesion, but the lumbar segments 
are affected by secondary degeneration. 

Sensation is impaired at a very early period of the dis- 
ease, often from the first moment of onset. In fact, in the 
cases of traumatic origin loss of power and loss of sensa- 




Fig. 80. — Seconda- 
ry Ascending and 
Descending De- 
generation follow- 
ing a Transverse 
Lesion in the Up- 
per Dorsal Cord. 
(Struempell. ) 



ACUTE MYELITIS. 315 

tion are effected almost instantaneously, so that a person 
thus afflicted feels not only that his legs are powerless, but 
that they appear to be dead as well. Anaesthesia exists 
in all the parts that are supplied by nerves coming off be- 
low the site of the spinal lesion, and in the typical cases all 
forms of sensation are equally involved. Thus we gener- 
ally find that a spastic paraplegia is associated with the 
loss of touch sense, of pain sense, of thermal sense, and of 
muscular sense in the affected parts. As the anaesthesia is 
strictly dependent upon the conduction of impulses inward 
through the posterior roots of the cord, the extent of the 
anaesthetic area will naturally give us a sufficient clew as to 
the upper limit of disease in the spinal cord. (See pages 
277 and 278.) At the upper limit of the anaesthetic area a 
small zone of hyperaesthesia, as a rule, begins. This is 
evidence of the fact that in the segment supplying the hy- 
peraesthetic area there is a condition of irritation affecting 
the posterior spinal root-fibres, but not the chief morbid and 
destructive process. Above the hyperaesthetic area sensa- 
tion may be expected to be entirely normal. In other cases, 
instead of a distinct hyperaesthesia we have a girdle sensa- 
tion, which also marks the level between the normal and 
diseased segments. 

The state of the reflexes helps us also to determine the 
area involved. If the lesion is in the cervical region all 
the reflexes of the upper extremities are destroyed, those 
in parts below will be exaggerated. If the lesion is in the 
dorsal region the reflexes connected with these segments, 
such as the abdominal and epigastric reflexes, will be lost 
and the lower reflexes will be increased. If the lesion is in 
the lumbar region the knee-jerk will be lost and the ankle 
clonus will be absent also. In some cases in which there 
is a very narrow band of inflammation these reflexes may 
behave differently and may give one a direct clew as to the 
exact extent of spinal inflammation. Thus in one case un- 
der my observation the knee-jerk was lost, but the ankle 
clonus was present. This, taken in conjunction with a line 
of anaesthesia showing an involvement of the lower dorsal 
segment, proved that the upper lumbar region was slightly 
involved, but that the lower lumbar and sacral segments 



3l6 THE NERVOUS DISEASES OF CHILDREN. 

were not directly implicated by the disease. Since dorsal 
myelitis is the most frequent form, it is also common to 
have exaggeration of the reflexes associated with spastic 
contractures of the legs. 

The electrical reactions will vary according to the seg- 
ments involved. In cases of cervical lesion the reaction of 
degeneration will be found present in many, if not all of the 
muscles of the upper extremities, at least after the lapse of 
a few days or weeks ; those in the lower extremities will 
remain unaltered. But if the lesion is in the lumbar seg- 
ments the reaction of degeneration will be present in the 
muscles supplied by nerves coming off from the diseased 
area. In cases of cervical and dorsal myelitis we may, 
therefore, expect entirely normal electrical reactions in the 
lower extremities, however thoroughly paralyzed these 
parts may be. 

A few other symptoms must be noted which are ex- 
tremely characteristic of acute myelitis and often serve to 
reveal the disease when other symptoms in the case have 
left the diagnosis in doubt. In all such cases of myelitis, in 
whatever region the lesion may be, the vesical and rectal 
reflexes are disturbed or completely abolished. The re- 
sult of this is retention of urine, with possible overflow, or 
constant dribbling of urine, and either retention of stool or 
involuntary defecation. Since the centres for these reflexes 
are in the lowest portion of the cord, and every part of the 
cord must be intact if such sensation is to be conducted to 
the higher centres, we can understand why these symptoms 
should be present, whether the lesion be in the lumbar, in 
the dorsal, or in the cervical segments. Loss of sexual 
function is hardly to be mentioned in cases of myelitis in 
children ; but priapism, due to irritation of the spinal sexual 
centre, is not infrequently present in children, and is some- 
times a very annoying symptom. Involuntary spasmodic 
twitchings occur in nearly every form of myelitis. I know 
of no symptom which is on the whole more characteristic 
of spinal lesions, and which often serves as a differential 
symptom between supposed functional and spinal paralysis. 
This spasmodic cramp is evidently due to an irritation of 
the normal ganglion cells, and the irritability is often so 






ACUTE MYELITIS. 317 

great that the mildest form of sensory impulse is sufficient 
to elicit such involuntary spasms. Under these circum- 
stances the mere touch of the paralyzed part — of a toe, for 
instance — is sufficient to produce contraction of the entire 

limb. 

Trophic disturbances are exceedingly common and 
much to be feared. Bed-sores are easily developed in all 
parts on which pressure is exerted : under the shoulder- 
blades, over the sacrum, on the hips, and even over the 
internal malleoli or on the inner surfaces of the knees and 
thighs — in short, wherever parts touch, are pressed upon, 
or are pressed against each other. In the sacral region the 
constant wetting of the bed and the uncleanliness of the 
patient may increase the danger and size of bed-sores ; and 
while it is true that such bed-sores may occur even without 
any external irritation, they are greatly aggravated by the 
dribbling of urine or the involuntary evacuation of the 
bowels. These bed-sores begin, as a rule, as a mere redden- 
ing of the skin ; the epidermis is soon worn away, the 
cutis is bared, this too disappears, and gradually the ulcer 
may eat away all the subjacent parts until the bone itself is 
laid bare, provided the patient lives a sufficient period of 
time. 

Every form of acute myelitis may be accompanied by 
fever, which may vary between ioo° and 104 F. and higher. 
The fever is unquestionably due to the myelitic process, 
but is often increased, and sometimes maintained altogether, 
by the complicating conditions of myelitis. Such compli- 
cations are deep bed-sores with the absorption of putrid 
matter and the danger of phlebitis ; furthermore, the oc- 
currence of cystitis and pyelonephritis, which are not un- 
common. In cases of myelitis, in which fever suddenly 
increases, with chills, with deep remissions and sudden ex- 
acerbations, the probability of this fever being due to some 
pysemic process is very great indeed. As soon as the dis- 
ease has passed the acute stage the fever lessens and the 
temperature will remain entirely normal until some com- 
plicating condition is established. 

Whenever a majority of the above symptoms are pres- 
ent, the diagnosis of an acute (transverse) myelitis can safely 



318 THE NERVOUS DISEASES OF CHILDREN. 

be made. The modification of the symptoms if the mye- 
litis is not complete, or if it is subacute and chronic can be 
easily inferred from the preceding account. The question 
of greatest interest, in every case, is to determine what the 
origin of the myelitis may be. Idiopathic myelitis is, ac- 
cording to our present notions, scarcely conceivable, and 
here, as in so many other instances, it is better to say mye- 
litis from unknown cause than to concede that in some 
cases the origin is truly spontaneous. In cases occurring 
in children, without known cause, I am inclined to suspect 
slight traumatism. 

A child of eight years, a healthy, beautiful girl, while walking on a coun- 
try road, had a desire to urinate. Her mother urged her to do so on the 
road. The child hurried to one side, and in attempting to place its right 
foot on a stone not more than a few inches in height, lost its bal- 
ance and struck on the middle of the back. The child experienced pain at 
once, but was able to walk some little distance ; soon the power of its legs di- 
minished; it had to be carried, was put to bed, and within twenty-four hours 
had developed a most pronounced form of acute transverse myelitis. After 
three days anaesthesia was complete up to the umbilicus. The paralysis 
was absolute in the lower extremities. There was retention of urine and 
faeces, bed-sores were developed, and the child died from these complicating 
conditions within three weeks after the accident. 

In other cases traumatic injury is much more severe, and 
a complete destruction of the cord may be the result of in- 
jury to the spinal column. From the effect of concussion 
alone, without actual destruction of the bony parts surround- 
ing the cord, an acute myelitis may result. The myelitis 
which occurs in connection with tumors of the cord, with 
tubercular affections of the meninges, need not be separately 
considered, as it constitutes merely a part of the more seri- 
ous disease. The two forms of myelitis which are most 
common in children are those due to Pott's disease and to 
syphilitic infection ; but both these forms are so distinct 
and so important that they deserve special consideration. 

Acute myelitis does, however, occur in connection 
with other acute infectious diseases, such as typhoid, scar- 
let fever, small-pox, and the like. Some of these cases of 
supposed myelitis have probably been cases of multiple 
neuritis. Rheumatic or atmospheric influences (refrigera- 



ACUTE MYELITIS. 319 

tion), as a direct cause of myelitis in the child and in early 
youth, should be considered duly. A young girl, of about 
sixteen years of age, was brought to me from the South, 
with the following history : On a very warm day in early 
spring she had taken a warm bath and had sat down at an 
open window in the evening, immediately after the bath, 
with nothing but a light chemise to cover her body. She 
sat there for hours and fell soundly asleep. The next morn- 
ing she experienced considerable difficulty in the use of 
both upper extremities. Within a few days these became 
absolutely paralyzed, and the legs at the same time grew 
stiff and motionless. She has since that time, a period of at 
least six years, been suffering from the effects of this myeli- 
tis, and even now presents an atrophic form of paralysis of 
the right upper extremity, with slight involvement of the 
left, and with a complete spastic paralysis of both lower ex- 
tremities, with incontinence of urine and faeces, and with 
considerable disturbances of sensation, though the latter 
have been recovered from very much more than has been 
the paralysis or the atrophy. 

Refrigeration is an important factor in the causation of 
spinal diseases. We have recognized it as a possible factor 
in acute anterior poliomyelitis, and we must recognize it as 
an equally potent factor in some cases of acute transverse 
myelitis. 

Pathology and Morbid Anatomy. — The delicate 
structure of the spinal cord seems peculiarly liable to in- 
flammatory disease. The cervical and lumbar enlargements 
of the cord are less frequently the seat of such inflammation 
than the dorsal portion. The reason of this is not easy to 
explain, although it must in all probability be sought in the 
peculiarity of the blood-supply. 

There can be little doubt that if the blood-supply of the cord is interfered 
with, necrotic softening follows as in the case of other organs. In the case 
of one form of myelitis — that due to specific disease — the relation of the my- 
elitis to disease of the blood-vessels can be clearly demonstrated. Under 
these conditions the smaller vessels are blocked by thrombi, and the result of 
this obstruction is a necrotic softening of the surrounding parts. In the eases 
of traumatic myelitis the earliest changes are due to mechanical injury of the 
parts, with compression of the delicate structures of the cord by effusion of 



320 THE NERVOUS DISEASES OF CHILDREN. ' 

blood, and to the necrosis that follows such compression. It is more difficult to 
explain the exact manner in which myelitis is developed in the cases of a toxic 
character, unless we suppose that the chemically altered states of the blood 
produce coagulation of the blood and obstruction of blood-vessels, with the 
same result as in those cases in which these conditions are brought about by 
other disease of the blood-vessels themselves. The origin of myelitis from 
refrigeration and rheumatic influences in general cannot be satisfactorily ex- 
plained in this way, nor can any other plausible explanation be substituted. 
The myelitis which results from mere concussion without any visible anatom- 
ical changes must be explained on the supposition that minute changes in 
the gray and the white matter are present, such as were found in the spinal 
cords of animals experimented on by Schmauss. 

If the exact mode of origin of various forms of myelitis 
is still unknown, the morbid anatomy is no longer a matter 
of doubt. As for the macroscopical appearance of myelitis, 
the cord so diseased is generally surrounded by the hyper- 
asmic meninges, and the cord itself, if inflammation is recent, 
may appear to be congested and slightly swollen. The dis- 
tinction between the white and the gray matter is often not 
so marked as in the normal cord. There is, furthermore, a 
change in the consistence of the cord, which may be either 
slightly softer than normal or else so diffluent that as soon 
as the pia is cut open the cord flows out like creamy pus. 
This is the condition often found on post-mortem exami- 
nation ; in all probability the cord is not nearly so soft 
during life, but, like other necrosed tissue, softens consid- 
erably immediately after death. In acute myelitis minute 
hemorrhages are extremely frequent, and the altered cord 
may present the appearance of red softening. There is 
every degree of change between simple red softening and 
the condition of hemorrhagic myelitis in which the extrava- 
sation of blood, being considerable, for the time obscures 
all other changes. If the blood has been exuded for some 
time before death its color may have changed, and the con- 
dition be that of yellow softening. We also may discern a 
condition of white softening in which the white matter has 
become diffluent without any admixture of blood. If ex- 
amined microscopically the cord is found to contain ample 
evidence of inflammatory changes. Among these are dilated 
blood-vessels, with leucocytes, granules of myelin, and, fur- 
thermore, bodies well known as corpora amylacea. The 



ACUTE MYELITIS. 321 

softened tissue also contains axis cylinders in various states 
of disintegration. 

In many cases of diffluent myelitis these changes of the individual ele- 
ments of the cord are the only ones that can be distinctly made out. But in 
the parts directly surrounding the focus of most intense inflammation further 
changes can be made out after proper hardening and staining with the various 
dyes. On such sections the blood-vessels will be found dilated, and innumer- 
able leucocytes can be seen in the vicinity of such vessels. The nuclei of the 
smaller arteries and capillaries will be found to be enormously increased and 
in a state of proliferation. The sheath of the blood-vessels is very much 
distended, blocked in part by the coagulation of blood, and round blood- 
corpuscles may even be found in the adjacent tissue. 

In the gray substance the large nerve-cells are swollen and granular, 
many of these granules showing distinct evidences of degeneration. The proc- 
esses of the cells are either shrivelled up or entirely lost, the contour of the 
cells less distinctly defined, and changed from the polygonal form to spher- 
ical or oval-shaped bodies. The neuroglia of the gray matter will appear 
denser than under normal conditions. In the white substance similar 
changes will be found in the blood-vessels and in the interstitial tissue. The 
white substance often has a distinctly fibrous appearance, containing many 
spider-cells or cells of Deiters. The white nerve-fibres themselves undergo 
degeneration. The axis cylinders are irregular, swollen, and often trans- 
versely divided. In some cases the entire nerve-fibre is destroyed or disin- 
tegrated and the space once occupied by such fibres is left vacant or occupied 
by granular matter. In some cases of myelitis the nerve elements are more 
intensely affected than the interstitial tissue, while in others the changes in 
the interstitial tissue are the more prominent feature in the cross-section, and 
the nerve-tissue has evidently been destroyed secondarily. In transverse 
myelitis the changes may be distributed equally through the entire cross-sec- 
tion ; in other cases the changes may be more intense in the gray than in the 
white matter, and in some more intense in the ventral half than in the dorsal 
half. In cases of meningo-myelitis, particularly in those of traumatic or 
specific origin, the most marked changes are near the periphery. Here the 
pia will appear thickened and the morbid changes can be traced along the 
connective tissue passing from the pia into various portions of the cord. If 
the myelitis is of the disseminated order, small foci of disease may appear in 
various portions of the cross-section and in various segments of the cord, 
intervening parts maintaining a tolerably normal appearance. If the myelitic 
changes are most prominent in the vicinity of the central canal and the parts 
surrounding it, we speak of a central myelitis ; but it is rare to find such 
central myelitis without some additional symptoms of a diffuse inflammatory 
process. The nerve-roots in connection with the inflamed segments are. as 
a rule, altered, and will present appearances somewhat similar to those found 
in the white matter of the cord. The vessels are dilated, the nuclei and the 
tissues about these vessels exhibit various degrees of proliferation, the myelin 

21 



322 THE NERVOUS DISEASES OF CHILDREN. 

is disintegrated, and the axis cylinder either swollen or distorted ; but these 
degenerative changes can, as a rule, be traced only a short distance from the 
diseased cord. 

Secondary changes follow upon the area of inflammation, and the tracts 
will be affected in an upward or downward direction, according to the direc- 
tion in which they transmit impulses. Thus, after a transverse myelitis the 
lateral columns will degenerate downward throughout their entire extent, but 
an ascending degeneration will occur in the parts that transmit impulses in a 
centripetal direction. (Fig. 80.) Among those exhibiting ascending degen- 
eration are the posterior columns, the cerebellar tract, and the antero-lateral 
ascending tract. The inflammation spreads a short distance upward and 
downward by contiguity, and those parts which one would suppose to be 
subject to descending degeneration only may be affected for a short distance 
above the lesion ; but such changes are of a distinctly inflammatory charac- 
ter and altogether different from the purely secondary changes, which rarely 
offend against physiological principles. These degenerations are, as a rule, 
developed very promptly after a transverse lesion, and often continue to exist 
after the initial inflammation has pretty well disappeared. 



Whether fibres that have once been seriously altered, 
or even destroyed, can ever regain their function or can 
grow anew, is a matter of serious doubt, and yet recovery 
takes place in a fair number of cases in which absolute paral- 
ysis, with signs due to descending degeneration, had existed 
for a number of months, or sometimes for a year or more. 
We must suppose in such cases that some fibres were so 
little altered that when the inflammatory products were ab- 
sorbed they still retained the power of conduction, and it is 
more probable that such fibres may be restored to absolute 
health than that entirely new fibres can be formed within a 
nerve-sheath, or that destroyed fibres can be replaced by 
new ones. 

Differential Diagnosis. — The more or less acute on- 
set, the often sudden loss of power, the rapid spread of anaes- 
thesia, the permanency of all these symptoms, together with 
the retention of urine and faeces, and the flaccid and atro- 
phic symptoms at the level of the injured part, together 
with the spastic symptoms in the parts supplied from seg- 
ments below the level of the lesion — all these symptoms 
will leave little doubt of the diagnosis of acute myelitis. In 
addition to this the etiological factors in the case — the occur- 
rence of traumatism, a preceding syphilitic infection or pre- 



ACUTE MYELITIS. 323 

ceding bone disease, or marked rheumatic influences — will 
help to corroborate the diagnosis. 

Acute myelitis may resemble hemorrhage of the cord, but in cases of hem- 
orrhage the onset is more sudden than in cases of acute inflammation, all the 
symptoms being developed within a very few minutes. There is, as a rule, 
too, much more pain than in cases of myelitis ; but hemorrhage is frequently 
enough the first stage of a myelitis, and if symptoms indicating a spread of dis- 
ease follow upon what is supposed to be an initial spinal hemorrhage, it is fair 
to conclude that a myelitis haemorrhagica has followed upon the initial extrava- 
sation of blood. Direct injury to the spinal column may be another factor 
tending to corroborate the diagnosis of hemorrhage. 

A rapidly ascending myelitis may suggest the acute ascending (Landry's) 
paralysis, but in cases of myelitis the progress will be clearly from the level 
of the first injury, and is not apt to attack the parts in succession from below 
upward, including the trunk, as in cases of Landry's paralysis. In ascending 
myelitis, moreover, sensation is disturbed from the start, and all the trophic, 
as well atrophic, symptoms are much more characteristic of a myelitis than 
they are of Landry's paralysis. But if the myelitis begins in the lumbar por- 
tion of the cord, and gradually spreads upward, the difficulties of diagnosis 
may be extremely great. Landry's paralysis is unusually rare in children, 
whereas myelitis is relatively frequent. 

The distinction between meningitis and myelitis is not of 
great practical importance, for meningitis is rarely present 
without some involvement of the cord, and if the symptoms 
are purely meningeal, they are generally associated with 
other symptoms pointing to a wide-spread affection. A pri- 
mary spinal meningitis is a great rarity, except as a part of 
cerebro-spinal disease or after disease or injur)- of the spi- 
nal column. The involvement of the meninges in a given 
case will be indicated by considerable pain in loco morbi, 
and by the presence of distinct neuralgic pain along the 
nerves emanating from the diseased portion of the cord. 

The question at times arises whether a case is one of myelitis or multiple 
neuritis. In the latter symmetrically located pains are a more prominent symp- 
tom, trophic disturbances are not so marked as in myelitis, and the symptoms 
never include vesical and rectal disturbances. Moreover, we never have that 
combination of paralytic and spastic symptoms which we so frequently find 
in cases of myelitis. In cases of multiple neuritis affecting all four extremities 
the symptoms in all the extremities are entirely the same, whereas in cases of 
myelitis they would be of the flaccid order, say, in the upper extremity, and of 
spastic order in the lower extremity. 



324 THE NERVOUS DISEASES OF CHILDREN. 

A more difficult task it is to distinguish between a mye- 
litis and hysterical paralysis, and yet a careful examination 
of the patient should reveal important points of diagnosis. 
Thus, in hysterical paralysis, legs that cannot be used in 
standing or walking may be moved freely in bed ; the 
rigidity is not so marked as in cases of myelitis, and if pres- 
ent can be more easily overcome, as a rule, than in myelitis. 
Bed-sores are rarely present in hysteria, and anaesthesia, if 
present, is anomalous in distribution. The reflexes, too, are 
not so distinctly exaggerated in hysteria as they are in cases 
of myelitis. If the symptoms should point to a lumbar af- 
fection, the lack of atrophy and the persistence of the knee- 
jerks will help to differentiate the hysterical paralysis from 
a spinal paraplegia. The bladder and rectal symptoms are 
also not so marked in hysterical as in myelitic cases. The 
very suddenness of the onset in hysterical cases, the fact 
that the paraplegia is frequently due to a sudden fright, or 
a deep emotional condition, may also point to hysteria rather 
than to myelitis. But in all such cases the fact that hysteri- 
cal subjects may suffer from organic lesion should be borne 
in mind. 

Prognosis. — The prospects in cases of acute myelitis 
will vary according to the level affected. Cervical myelitis is 
naturally a more serious disease than myelitis of lower por- 
tions of the cord, for in the former an extension upward to 
the respiratory and cardiac centres constitutes one of the 
grave possibilities of the case. In these, as well as in dor- 
sal and lumbar myelitis, the danger to life arises chiefly 
from the complicating conditions, particularly from bed- 
sores, from cystitis, and pyelo-nephritis. The earlier these 
symptoms set in the graver the prospect of the case ; but 
not a few of such cases get well in spite of all compli- 
cations, and if the myelitis can be proved to be due to spe- 
cific disease, or to some other form of mild toxic infection, 
recovery is more probable than in the traumatic cases of 
myelitis or those in which the etiological factor was en- 
tirely unknown. 

The prognosis will also vary according to the intensity 
of the affection and the extent of cord involved. If all the 
symptoms are developed rapidly, then become stationary 



ACUTE MYELITIS. $2$ 

and show not the slightest sign of improvement for weeks 
or months, the probability of spontaneous recovery is ex- 
tremely slight ; but any improvement which sets in, either 
in the form of diminution of anaesthesia, of the disappear- 
ance of bed-sores, or of a slight gain in motion, is a hopeful 
sign of greater improvement later on. I have myself seen 
complete recovery in cases of myelitis in which the palsy 
was absolute for a period of nearly six months, with 
marked contractures and increase of the reflexes and with 
slight vesical symptoms, but I cannot recall a single case 
in which complete recovery set in if deep bed-sores de- 
veloped at an early day, and marked cystitis appeared very 
early in the disease, the only exception to this rule being 
in cases of distinct specific myelitis. 

Treatment. — In the treatment of myelitis the follow- 
ing plan should be pursued if the patient is seen during 
the acute stage. An ice-bag should be applied to the 
greater part of the spinal column ; counter-irritation may 
be used, but the danger of trophic changes in the skin 
should be remembered, and such trophic changes should 
not be encouraged or started up by an excessive use of 
counter irritants. The patient should be placed absolutely 
at rest, if possible with some form of extension. His bow- 
els should be thoroughly purged, best by the use of calo- 
mel, and the bladder catheterized by careful hands and 
watched for the first signs of a cystitis. (If cystitis should 
develop, no time should be lost, even during the acute stage, 
in beginning the usual treatment for such conditions.) All 
these measures will tend, first, to make the patient more com- 
fortable, and, secondly, they will surely lessen the danger 
from complicating conditions. The diet should be of a mild, 
non-irritating kind, and the kidneys should be encouraged 
to greater activity. Under such conditions the administra- 
tion of small doses of digitalis or of the acetate of potash 
will be quite in order. Ergot was recommended years ago 
by Brown-Sequard, and may be administered with the idea 
of limiting the area of inflammation. 1 cannot say that 1 
have ever seen any direct results from ergot, but it seems 
to do no harm, and considering the seriousness of the dis- 
ease it may well be tried. In cases in which there is reason 



326 THE NERVOUS DISEASES OF CHILDREN. 

to think that there is much inflammatory exudation, and 
particularly in those of specific origin, the administration 
of the iodides is quite in order, or of the mercurials and 
iodides combined ; but if mercurials are exhibited it is use- 
less to give them in any other way than by inunction. 

The main objects are to prevent the serious complications 
so common in these diseases, and to give the diseased or- 
gans a fair chance of spontaneous recovery. It is of the 
utmost importance, therefore, in cases of myelitis, to keep 
the patient absolutely clean — a task not so easy in view 
of the frequent dribbling of urine and of the involuntary 
passage of fseces. The child should be placed upon a 
water-bed. Nurses should, invariably, receive instructions 
to keep the bed-linen absolutely smooth, and to promote 
this end it will be best to have the bed dusted very liberally 
with some slightly aseptic powder. In the case of female 
patients, pads should be put in place to catch the dribbling 
urine and to prevent its soaking adjacent parts ; in the 
case of boys, urinals should be used from the Start. It is 
a common practice with me to order the patient's posi- 
tion in bed to be changed at least every hour, so that no 
one part is pressed upon for too great a length of time. 
If in spite of all these precautions bed-sores should form, 
these should be treated according to the best surgical prin- 
ciples. Latterly I have been in the habit of dusting the 
sores with dermatol or aristol and covering them com- 
pletely with light antiseptic dressing, shielding the dress- 
ing as well as may be from contamination by urine or 
fasces. It is better to change the dressing frequently than 
to allow any infection of the sores through uncleanliness. 
In hospitals or among the poorer classes less expensive sub- 
stances, such as bismuth, may be used ; and if cystitis has 
been set up, the bladder should be washed several times a 
day with some weak antiseptic solution. 

After the symptoms of the acute stage have been suc- 
cessfully treated, and the patient has passed into a more 
or less chronic condition, the question arises as to the 
proper treatment of this latter stage of the disease. I am 
in favor of making the attempt again and again of affecting 
the focus of inflammation by the administration of the 



ACUTE MYELITIS. 32 J 

iodides. In nine cases out of ten this will be unavailing*, 
and yet there is no good reason why the effort should not 
be made. But the possibility of recovery should be well 
weighed in the scales as compared with the gastric disturb- 
ance which these drugs so often excite. The nutrition of 
the child must be maintained at all odds, and it is far better 
to abandon the iodides than to permanently impair the as- 
similation of food. If the iodides cannot be given, inunc- 
tions of the oleate of mercury or of the usual mercurial 
ointment should be substituted for the iodides. Counter- 
irritation may be attempted, either by the cautery, by blis- 
tering, or by mere cupping ; but little direct good is to be 
expected from these remedies. The child should be kept 
absolutely at rest, and the spinal column should be disturbed 
as little as possible. Electricity applied to the spinal cord 
is of very doubtful utility, but there is all the more reason 
for using it in the treatment of the paralyzed parts. It 
does excellent service here as a form of exercise for the 
maimed limbs. Massage has very much the same, and, I be- 
lieve, a better, effect than electricity. It tends, in addition, 
to maintain the nutrition of the parts, and to overcome the 
tendency to contractures, which so frequently give rise 
to the most disagreeable symptoms in these cases ; but nei- 
ther electricity nor massage should be pushed if the invol- 
untary spasmodic contractions become more frequent, as 
they often do in consequence of these measures. These 
contractions are not harmful, but are extremely irritating to 
the patient, and if inordinately increased may disturb his 
rest, and thus interfere with the general nutrition. 

As soon as the child has sufficiently recovered, it is of 
the greatest importance to give it all the fresh air possible, 
and, if necessary, to provide it with a wheeled chair, so that 
it can be given its regular outing. Its diet must be care- 
fully looked to, and all unnecessary excitement should be 
avoided. Tonic measures may be employed ; and if the 
child is in the charge of a competent person give lukewarm 
baths, followed by cool or cold douches of the spine, and 
let this be done before the massage is given. Iron, quinine, 
or arsenic may be administered for their general tonic ef- 
fect. Strychnine may be given in very small doses in those 



328 THE NERVOUS DISEASES OF CHILDREN. 

cases in which there is reason to think that the substance 
of the cord has not been absolutely destroyed, and that the 
function of the diseased parts could be increased by the use 
of this drug. I know that it is an extremely popular drug 
in these diseases with all physicians who are not specialists, 
but I have found it to be a double-edged weapon. If it in- 
creases nerve conduction for a time, such improvement is 
very apt to be followed by a further diminution of func- 
tion, and in other cases again it produces annoying mus- 
cular contractions, which are as disagreeable as those that 
result from an excessively strong electrical current. In 
these cases, too, the physician who watches his patient 
carefully, and does not attempt to do too much, will suc- 
ceed far better than he who is continually meddlesome and 
ever anxious to change treatment. 

TRAUMATIC INJURIES OF THE SPINAL CORD. 

The direct results of jumping, of falls, and of injury due to falling weights 
are, on account of the more delicate nature of the spinal vertebrae, more seri- 
ous in childhood than in later years. A fall downstairs, or a simple fall out of 
bed, or a mild blow inflicted by another child, may be a sufficient cause to start 
the symptoms pointing to actual injury of the spinal cord. 

The symptoms of spinal-cord injury may be extremely varied. I do 
not propose now to discuss those cases in which traumatism is the remote 
and questionable cause of a subacute or chronic form of spinal disease de- 
veloping months, or even years, after an accident ; but I wish particularly to 
direct attention to those cases in which the traumatic injury is followed im- 
mediately, or within a period of a few weeks, by symptoms which point to 
direct injury. In the severest form of traumatic injury to the cord there is 
immediate paralysis both of the motor and sensory functions. Inasmuch as 
the injury more frequently involves the dorsal and lumbar portions, a spastic 
paraplegia with anaesthesia of the lower extremities, with loss of vesical and 
rectal control, constitute the chief symptoms which may be developed within 
a few minutes or within a few hours after the injury. 

If examined more in detail we can determine by the character of the 
symptoms, first, the exact portion of the cord involved by the injury, and sec- 
ondly, the amount of injury done at any level. The question of the exact 
extent of the injury up and down the cord is determined by the parts paral- 
yzed, and more particularly by the extent of the anaesthesia. These cases are 
indeed well calculated to illustrate the principles of spinal localization. In 
cases of cervical lesion the upper as well as the lower extremities are in- 
volved. In the upper extremities the paralysis is of an atrophic order, while 
spastic paralysis is present in the lower extremities. The anaesthesia in- 



ACUTE MYELITIS. 



329 



volves both the upper and lower extremities as well as the trunk, to or 
from a level supplied by the nerves coming off from the injured segment. 
In cases of cervical injury the sympathetic may be involved. In cases of in- 
jury to the dorsal region, which are the most frequent, the arms are not af- 
fected, the lower extremities are in a condition of spastic paralysis, the 
bladder and rectum may be involved, and the anaesthesia extends from be- 
low upward to a level corresponding to the segment or segments injured. 
The band of hyperesthesia, or the upper level of the anaesthesia, will cor- 
respond to the upper limit of injury. If the 
injury has been done to the lumbar enlarge- 
ment, the paralysis of the legs is of a flaccid 
order, the reflexes are diminished or lost, the 
vesical and rectal reflexes lost, and the areas 
of anaesthesia will vary according to the seg- 
ments involved. Injuries to the cauda equina 
are of particular interest because of the pecul- 
iar character of the anaesthesia, upon which 
alone an accurate diagnosis can be based.* 

The exact study of the symptoms will 
often help us to determine the upper as well 
as the lower limits of injury. Thus in one 
case which I have had occasion to observe, 
there was spastic paralysis with slight dimi- 
nution of the knee-jerks, but presence of 
ankle clonus. There was spasmodic twitch- 
ing of the legs, loss of vesical and rectal 
reflexes, as well as a tendency to bed-sores. 
The difference in the behavior between the 
knee-jerks and the ankle clonus proved con- 
clusively that the injury had slightly involved 
the upper lumbar segments, chiefly the lower 
dorsal region, but that every part of the cord 
below the upper lumbar segment had escaped 
injury, or else the ankle clonus would surely 
not have been present. In determining the 

extent of the cross-section involved, a very important question, and one which 
helps us to decide whether a complete crush has taken place or not. we must 
keep the physiology of the cord in mind, and endeavor to make out whether 
the anterior horns as w T ell as the lateral or the posterior columns have been 
involved. Thus, in a case in which the lesion was in the lower dorsal region, 
and in which there was complete anaesthesia with marked spastic paralysis, I 
found that the muscles of the back showed, after a lapse of weeks, neither a 
tendency to atrophy nor to changes in electrical reactions. I argued from 
this, and correctly too, as the event proved, that the chief injury was done 
to the posterior and lateral portions of the cord, and that the ventral portions 
had entirely escaped. 

* See the paper by Starr in American Journal of the Medical Sciences, 1 r.h , 




Fig. 81.— Sketch of Section of 
Spine in a Case of Fracture 
Dislocation of the Seventh 
Cervical Vertebra. (After 
Thorburn. ) 



330 THE NERVOUS DISEASES OF CHILDREN. 

Another aid to accurate diagnosis of the lesion will be found in the sen- 
sitiveness to pressure over the spinal column at the seat of injury. The 
parts that are sensitive should correspond to the segment of the cord which 
an examination of the patient has shown to be diseased, and before coming 
to any definite conclusion the physician should remember the relation of the 
external parts to the segments of the cord, as given in Figure 64. If these 
two sets of facts do not accurately correspond, the preference should, to my 
mind, be given to the seat of injury as determined by the study of the 
paralysis and the anaesthesia ; but if the level determined in this way is not 
far distant from the seat of pain on pressure, both should be included within 
the area to be operated upon. 

The course of the disease will depend largely upon the region affected 
and upon the extent of injury done. Injuries to the cervical region are, on the 
whole, more serious than those to the dorsal and lumbar portions of the 
spinal cord. Injury to the lumbar spine is generally followed by more serious 
symptoms than is the case after dorsal injury. If the initial symptoms in- 
dicate a comparatively slight lesion at any level, the progress of the disease is 
apt to be more favorable than if a complete or nearly complete crush of the 
cord has occurred. If the symptoms show a tendency to improvement after 
a few weeks, or after a month or more, the possibility of complete recovery 
may be considered ; but if they remain stationary for a long period without 
the slightest indication of improvement, actual recovery is rare, unless re- 
lieved by operation. 

The danger to life is greater in the cervical cases, in which the proximity to 
the vital parts is of much importance ; and in severe cases of dorsal or lumbar 
injury the complicating conditions, such as cystitis and bed-sores, may bring 
about a rapidly fatal issue. After an initial injury that is relatively slight, de- 
generation may set in, which will be characterized by the onset of rigidity and 
contractures, and from the onset of these symptoms the prognosis as regards 
complete recovery may become very much graver. 

Pathology.— The actual anatomical lesions in traumatic injuries of the 
spinal cord may vary greatly. Even without injury to the vertebral column 
hemorrhage may occur from the effect of the shock, and this may be either 
epidural or subdural. The probability of the seat of the hemorrhage will have 
to be argued from the general character of the symptoms. In persons whose 
arteries are fragile, hemorrhage is much more likely than in persons whose 
vascular system is entirely normal. Persons with syphilitic disease will, 
therefore, be much more liable to traumatic hemorrhage than those not so 
affected. If the hemorrhage is considerable, whatever its location may be, 
the cord will suffer from compression and may undergo softening unless the 
blood that is exuded is rapidly absorbed. In all cases, inflammatory prod- 
ucts, which may be tinged by blood, will be found at the seat of injury. At 
times, in spite of a sudden paralysis developed immediately after an injury, 
no tangible lesion can be discovered at the time of operation or on the post- 
mortem table. We must then suppose either that the evidences of the initial 
lesion have disappeared, or that the traumatism has resulted in functional 
changes, or in such, at least, as are beyond the discovery by our present 



ACUTE MYELITIS. 33 I 

methods. If examined months or years after the initial injury, the cord may 
present nothing but the ordinary symptoms of chronic myelitis with consider- 
able shrinkage and wasting of the entire substance of the cord. Such wast- 
ing may be at times more marked in the white columns, at other times more 
distinct in the gray matter. In addition to the local changes found, the cord, 
if examined carefully, will reveal ascending and descending degenerations in 
accordance with the intensity of the process at any given level. I have seen 
several cases of spinal injury with severe spinal symptoms in which at the 
time of the operation no tangible changes were found in the cord, but the 
cord was evidently compressed by inflammatory exudations that had collected 
between the bone and the dura. Adhesions also form under these conditions 
between the dura and the surrounding parts. The impairment of function is, 
therefore, due to extra-spinal conditions. The breaking up of such adhesions 
and the removal of such inflammatory products are followed by improvement 
in the condition of the patient, if such removal is effected within a relatively 
short period of time after the accident. 

Treatment. — In cases of traumatic injury of the spinal cord, absolute 
rest is essential. The case may be treated in every respect for the first few 
days as though it were a case of non-traumatic myelitis, that is, by applica- 
tion of cold to the spine and by extension. As soon as the condition of the 
patient will permit, a careful examination should be made in order to de- 
termine the amount of injury done. The details of the treatment will not 
vary from that advised in cases of myelitis. The old habit of using the 
actual cautery, of blistering, and the like, is not to be recommended, for 
little good can follow it. As was said in the case of myelitis, the danger of 
trophic disturbances in the skin is great enough without such additional en- 
couragement. The most important question that arises is whether anything 
can be done for the patient by surgical means. There is still much hesita- 
tion on the part of physicians and surgeons in this respect. 

If the patient is in good general condition, and is able to stand the shock 
of the operation, the advisability of such surgical interference should be con- 
sidered purely upon the merits of the case, and if the evidence points to the 
fact that injured bone is pressing upon the cord, or that severe hemorrhage 
has occurred and that there is danger of permanent harm to the cord, the sur- 
geon should be permitted to expose the injured region. This can be done 
with considerable impunity by competent surgeons at the present day, and. 
as I have said, should be done as early as practicable after the injury. The 
cases in which surgical interference is useless are those in which all the 
symptoms indicate absolute crush of the cord, or in which the rapid devel- 
opment of all the symptoms points to a probably fatal issue ; or those in 
which so long a period of time has elapsed since the injury that there is no 
good reason to believe that the conditions can be relieved by operation. I 
have advised operations upon the spinal column in four cases, two of which 
were in children under the age of fifteen. The results were satisfactory, al- 
though neither one attained complete recovery ; but I attribute this to the 
fact that the cases were sent to me at too late a period after the accident. 
The operations are not nearly so dangerous as those upon the brain, and 



332 THE NERVOUS DISEASES OF CHILDREN. 

much encouragement may be derived from the statistics of various surgeons 
who have not lost a single case of spinal operation for traumatic injuries. If 
for some reason or other the operation has not been performed and the pa- 
tient is left with a chronic spinal disease, the attempt may be made to bene- 
fit the patient by the administration of the iodides ; and the crippled con- 
dition of the extremities may be somewhat improved by ordinary surgical and 
orthopedic measures, such as have been frequently referred to in the discus- 
sion of myelitis and other forms of palsy. 

LANDRY'S PARALYSIS. 

Acute ascending paralysis (Landry's paralysis) is extremely rare in child- 
hood. It has been considered in connection with multiple neuritis, from 
which it is to be differentiated. It comes on after acute infectious diseases 
and from exposure to cold ; it is supposed by some to be due occasionally to 
syphilis. The paralysis begins in the legs, spreading from one to the other, 
then it involves the muscles of the abdomen, the thorax, the upper extremi- 
ties, and may spread to the muscles of the pharynx, larynx, and eyes. The 
paralysis is generally flaccid, sensation may be disturbed, and the reflexes are 
lost. The electrical conditions are generally disturbed, but not to the extent 
we find in poliomyelitis or in neuritis ; fever is sometimes present, sometimes 
absent. Enlargement of the spleen has been noted. The case may prove 
fatal within several days or a week. Some of the cases go on to recovery ; 
the paralysis, as a rule, disappearing first from the upper extremities. 

It is questionable whether the disease affects the peripheral nerves only, 
or whether it involves the central nervous system. The probability is that 
in this, as in other toxic affections, any or all parts of the nervous system may 
be involved. 

BIBLIOGRAPHY. 

MYELITIS. 

Chavier et Fevrier : Revue de Medecine, December, 1888. 

Church : American Text-book of the Diseases of Children, 1894. 

Eisenlohr : Deutsche Med. Wochenschrift, 1890. 

Jacobi, Mary P.: In Keating's Cyclopaedia. 

Leyden : Zeitschr. f. kl. Med., Bd. I., 1880; Neurolog. Centralblatt, 1892, p. 

115. 
Moeli : Arch. f. Psychiatrie, Bd. XL, p. 757. 
Oppenheim: Berl. klin. Wochenschr., August 3, 1891. 
Raymond: Revue de Medecine, March, 1886. 
Schmaus : Compressionsmyelitis, etc. Wiesbaden, 1890. 

Other articles bearing upon this same subject are quoted in the chapters 
on Syphilis of the Spinal Cord, Compression of the Cord, etc. 



CHAPTER XVIII. 

SYPHILIS OF THE SPINAL CORD: SPECIFIC MYELITIS AND 
MENINGO-MYELITIS. 

In the adult, syphilis of the spinal cord can now be rec- 
ognized by a definite combination of symptoms. This is 
due to the researches of Erb, Gowers, Marie, Oppenheim, 
Siemerling, Hoffman, and others. In the child syphilitic 
disease of the spinal cord is not nearly so frequent as in the 
adult, but I do not hesitate to devote a special section to 
this subject, both because I think such cases can be easily 
overlooked and because they present a number of points in 
differential diagnosis which are of the greatest interest and 
which must be carefully considered if grave errors in diag- 
nosis are to be avoided. 

Symptoms. — In former days it was customary to make 
the diagnosis of syphilis of the spinal cord if the symptoms 
pointing to spinal lesion were irregular and would not fit 
into any of the ordinary types of spinal disease. As a mat- 
ter of fact the nature of the morbid process underlying 
these syphilitic spinal diseases is such that irregularities in 
distribution, and in the development of the symptoms, are 
very apt to occur, and yet we should be able to recognize 
the symptoms of syphilitic disease whether or not the pa- 
tient reveals, or we can prove, previous syphilitic infec- 
tion. 

In all but a few of the cases the onset of the disease is 
gradual. By degrees the legs or the arms, or both the 
lower and the upper extremities, which have shown some 
weakness, become paralyzed. One leg, or one arm, is at 
times more paralyzed than the other. This paralysis may 
be of the atrophic kind, but is much more apt to be of a 
spastic order. It is often associated with intense pain (par- 



334 THE NERVOUS DISEASES OF CHILDREN. 

aplegia dolorosa), or with anaesthesia — the anaesthesia and 
paralysis may be crossed (Brown-Sequard type). The re- 
flexes are generally increased, rarely absent. If the specific 
process is situated in the cervical segments there are atrophic 
paralysis with loss of reflexes in the upper extremities, spas- 
tic paralysis with rigidities and contractures in the lower 
extremities. The vesical and rectal reflexes may be inter- 
fered with. If the dorsal or lumbar segments are involved, 
the symptoms will closely resemble those following upon 
myelitis of the respective segments ; trophic disturbances 
may occur ; bed-sores may develop ; in short, we may have 
all the symptoms of a wide-spread spinal affection. It is evi- 
dent that if we wish to distinguish between specific disease 
of the cord and the various forms of acute or chronic my- 
elitis we must look for some distinct points of differential 
diagnosis. 

Erb, referring to be sure to the conditions in the adult, 
has established a type of spinal-cord disease Avhich he pro- 
posed to call syphilitic spinal paralysis. This special type 
bears the following characteristics : First, the usual symp- 
toms of spastic paraplegia, with its peculiar gait, carriage, 
and movements ; second, marked exaggeration of the deep 
reflexes ; third, muscular contractures, which are slight as 
compared with the exaggeration of the reflexes ; fourth, 
involvement of the bladder ; fifth, a slight yet distinct dis- 
turbance of sensation ; sixth, gradual onset of the disease ; 
seventh, a decided tendency to improvement. There is no 
doubt that this type of spinal disease, so well characterized 
by Erb, does occur. The same series of symptoms has been 
recognized by Rumpf, and since Erb's publication cases of 
this description occurring in children have been observed 
by Friedmann and by myself. But I have taken some pains 
to prove that there are other types, quite as frequent as this 
one, and that it is a great mistake to hesitate in making the 
diagnosis of spinal-cord syphilis unless the symptoms of 
Erb's type are present. It is the author's conviction that, 
if we wish to make a positive diagnosis of syphilis of the 
spinal cord, we should pay attention to the following points, 
and not exclusively to those presented by Erb as character- 
istic of the special type he has described. 



SYPHILIS OF THE SPINAL CORD. 335 

First, the most striking feature of syphilis of the spinal 
cord is the unusual distribution of the disease over the 
greater portion of the cord, involving, as it often does, the 
cervical and dorsal, as well as the lumbar enlargements. 
Second, the slight intensity of the morbid process at one level 
as compared with the extensive area involved, as evidenced 
by the preservation of some of the functions of the cord 
with complete loss of others. Third, the rapid dwindling 
of some of the symptoms and the very chronic persistence 
of others. Thus in some of my cases the anaesthesia lasted 
but a very short time, while the paralysis was recovered 
from with extreme slowness. Fourth, the very frequent 
history of other symptoms pointing to specific disease in 
the same or distant parts of the central nervous system. 

To emphasize these views let me state that I should be inclined to suspect 
specific disease of the cord if the patient presents symptoms of paralysis, 
whether they be of the spastic or flaccid character, and whether the contract- 
ures be slight or not, provided he furnish evidence of a morbid process affect- 
ing a very large part of the cord, and yet showing a relatively slight intensity 
at any given level of the cord. He may, for instance, exhibit the symptoms of 
extreme paralysis, spastic or atrophic, with partial or slight anaesthesia of the 
parts paralyzed, with little or no involvement of the bladder ; or, as often hap- 
pens, he may present traces of specific disease in other parts of the central 
nervous system. The chief difference, according to this, between the ordinary 
forms of myelitis and the specific diseases of the cord can be understood if 
we remember that the symptoms of an acute or subacute myelitis prove that 
the entire cross-section of the cord is affected almost simultaneously and to 
an equal degree, whence it follows that in such cases severe paralysis is likely 
to be associated with severe anaesthesia, with marked contractures, with abso- 
lute loss of vesical and rectal control, with serious trophic disturbances, and 
so on ; whereas, in the cases of spinal syphilis the morbid process invades the 
cross-section of the spinal cord partially and slowly. We may, therefore, 
find symptoms which point to a very marked affection of one or more of the 
systems of the spinal cord and to relative immunity from disease of the gray 
matter or other portions of the cord. Thus we may have extreme paralysis, 
but only slight ansesthesia ; or extreme loss of power, with relatively slight 
rigidity, as Erb pointed out. Furthermore, in the ordinary cases of myelitis, 
the symptoms point to a certain portion of the spinal cord at which the dis- 
ease is most intense, while in cases of syphilis of the spinal cord the clinical 
symptoms show that the disease involves a very large portion, if not the en- 
tire spinal cord, yet affects each single segment but relatively little. A still 
further aid to differential diagnosis is the very frequent involvement of the 
brain, at the same time that the majority of the symptoms point to disease of 



336 THE NERVOUS DISEASES OF CHILDREN. 

the spinal cord, or if these two sets of symptoms do not set in simultaneously, 
we frequently have in a patient who presents symptoms of a specific myelitis 
the history of a preceding illness in which the symptoms were of a cerebral 
rather than of a spinal character. (Unequal ocular, and, above all, unequal 
pupillary symptoms, are very common symptoms of cerebral syphilis.) The 
rapid and often unexpected recovery, as well as the relapses, help also to dis- 
tinguish these cases from the usual forms of myelitis. 

In view of the rarity of these diseases, or possibly of the 
failure to recognize them when they do occur, I wish to 
give a few typical cases. The first one I take from Fried- 
mann's article on relapsing, probably specific spastic, spinal 
paralysis in childhood. 

A boy, five years of age at the time of examination. Nine months before 
the birth of this child the mother miscarried with twins at the end of the sec- 
ond month of pregnancy. The birth of the boy was entirely normal, but 
the head was said to have been very large. Relative reduction of the size of 
the head in the next few months. Four weeks after birth a skin eruption 
appeared which covered the entire body; it was vesicular at first; later 
on it ulcerated, and terminated in desquamation. The child began to ex- 
hibit normal mental development, learned to talk at the end of one year, 
and began to teeth at the age of six months. At the age of three months 
there was distinct difficulty in moving the arms and legs. Three months 
later the right arm could be moved, and at the age of one and a quarter years 
all four extremities appeared to be entirely normal. In the second year, the 
child having learned to walk well, paralysis again gradually developed in the 
left arm, and disappeared once more after six weeks. From the second to the 
fourth year the boy was healthy, and passed through measles and diphtheria 
without any disagreeable sequelae. In the fourth year he complained much 
of headache, particularly in the occipital region, and gradually his gait became 
weaker and weaker, and he began to drag the right leg, frequently falling in 
the attempt to walk. There was slight difficulty in micturition and the legs 
were somewhat rigid. In this attack the arms were entirely free, and there 
was no history of spasms or convulsions at any time. The patient was in 
this condition when examined by Friedmann. The only other points of in- 
terest in the case were the very bad condition of the teeth, slight increase of 
the reflexes, and entirely normal sensation. 

Very recently J. Hoffmann has reported the case of a boy, 
who, at the age of twelve years, developed a typical spastic 
paraplegia, evidently due to hereditary syphilis, manifest 
signs of which appeared in the first years of life. 

The following case, observed by myself, presents many 
similar features : 









SYPHILIS OF THE SPINAL CORD. 337 

E. S , a girl, aged six years, born in this country, of German parents, 

was brought to my clinic in June, 1893. The mother gives a history of pro- 
tracted labor, but child was entirely normal ; began to cut teeth at six months, 
to stand and walk at the age of one year, and learned to talk well before she 
was two years old. 

The child had been perfectly well, with exception of mild attacks of 
whooping-cough and measles. At the age of five years the mother noticed 
that the child began to walk in a peculiarly stiff manner, and that its mental 
development was somewhat retarded. On examination we noted spastic par- 
alytic gait ; spastic paraplegia of lower extremities, more marked on left side ; 
left upper extremity slightly paretic and rigid ; both knee-jerks exaggerated ; 
triceps and wrist reflexes lively on left side. Pupils unequal ; left pupil dilated 
and does not react to light ; right pupil reacts sluggishly to light ; both pupils 
react sluggishly during accommodation. No sensory symptoms. 

The suspicion of syphilitic disease was strengthened by an examination of 
the mother. She has had two miscarriages ; three children died in early life. 
Five years ago (at the age of thirty years) she had left hemiplegia ; no loss of 
consciousness ; recovered in a few weeks ; has distinct " rheumatic " attacks ; 
gets dizzy while washing her face in the morning ; her pupils are unequal ; 
no reaction to light or during accommodation ; knee-jerks absent ; slight 
Romberg symptom ; no bladder trouble ; delayed sensory perception in the 
lower extremities. 

Thus we have a tabic, if not a purely syphilitic affection in the mother, and 
spinal syphilis in the child. 



Differential Diagnosis. — It seemed to me best to in- 
troduce most of the salient points of differential diagnosis 
in the description of the symptoms of the disease, and the 
comparison with myelitis was inevitable. On the distinc- 
tion between myelitis of the ordinary types and specific 
disease of the spinal cord, I need say nothing more. Syph- 
ilis of the spinal cord might be confounded with a spastic 
infantile palsy, particularly with a spastic diplegia and 
paraplegia resulting from meningeal hemorrhage occurring 
during the period of labor ; but in these latter cases the 
trouble can be traced distinctly to the earliest period of 
life, and there is never any history of relapses, and rarely of 
any marked improvement followed by relapse. Moreover, 
in the typical cerebral palsies the contractures are apt to 
be more extreme, and defective cerebral development is 
much more frequent than in the syphilitic cases coming on 
subsequently to the birth of the child. An acute infantile 
cerebral palsy might possibly simulate specific disease. 



333 



THE NERVOUS DISEASES OF CHILDREN. 



were it not for the far greater frequency of the hemiplegic 
form of paralysis in these cases than in the purely syphi- 
litic types of disease. Special difficulties might, however, 
arise in cases in which the acquired infantile hemiplegia 
would be proved to be due to syphilitic disease of the ar- 
teries, and under such conditions a combination of spinal 
syphilitic disease with cerebral disease would not be im- 
possible, although I have not yet come across a case of this 
description. Spinal syphilis in a child should not be con- 




Fig. 82. — Case of Multiple Cerebro-spinal Syphilis. Section through pons showing 
gummatous formation {g) in ventral portion, with considerable destruction of tissue. 
Weigerfs hasmotoxylin stain ; low power.* 

founded with hereditary spastic paralysis ; possibly the 
latter may be developed on a specific basis. 

Morbid Anatomy. — The anatomical changes in the 
spinal cord, due to syphilis, may be quite as varied in the 
child as in the adult. It is well known that syphilis is apt 
to cause disease of the blood-vessels. Obliterative endoar- 
teritis, with subsequent softening of the area supplied by 
the diseased vessel, is perhaps the best known anatomical 
process directly attributable to syphilis ; but a general ar- 

* Figs. 82-84 are reproduced by the courtesy of the editor of the New York Medi- 
cal Journal. 



SYPHILIS OF THE SPINAL CORD. 



339 



teritis is quite as frequent as an inflammation of the endo- 
thelium alone. Bruce has directed attention to changes in 
the adventitia (nodose periarteritis); moreover, veins are 
subject to syphilitic changes quite as often as the arteries 
are (phlebitis obliterans, Greiff). All these vascular changes 
are much more pronounced in the pial covering than in the 
substance of the spinal cord. 

The investigations of other authors, as well as my own, 




Fig. 83. — Section from Ventral Surface of Medulla Oblongata (high power), show- 
ing Infiltration of Pia and Substance of Medulla {a) and Typical Syphilitic Arteritis ; 
Marked Thickening of, and cellular proliferation in Intima (/) ; Narrowing of the 
Lumen ; Cellular Infiltration of Adventitia {b~). 



have shown that syphilis of the spinal cord is more often 
associated with a subacute or chronic meningitis, or me- 
ningo-myelitis, than with anv other process. The disease 
starts, as a rule, in the pia, and subsequently invades the 
spinal cord. There can be little doubt, however, that the 
changes may in some instances be developed in the reverse 
order. 

In the gross specimen we find the pia thickened and 



340 



THE NERVOUS DISEASES OF CHILDREN. 



often covered by a thick gelatinous substance. On micro- 
scopical examination this thickening is seen to be due to a 
proliferation of all the tissues. The cells are multiplied in 
number, the nuclei have increased, and the blood-vessels 
show the characteristic changes of specific arteritis. The 
walls of these vessels are thickened, all of the coats partici- 
pating to an equal degree. The process very often starts 
in the intima, but not invariably so, as was maintained some 
years ago by Heubner. The pia is generally adherent to 



<#& 




Fig. 84. — Section through a Portion of Dorsal Cord (Ventral Surface). Marked 
thickening of pia ; cellular infiltration of same, seen best in that portion which pro- 
jects inward ; infiltration of substance of cord. 

the spinal cord, and the cellular infiltration extends from it 
into the substance of the spinal cord. This invasion is a 
very gradual one, and may begin at almost any point of the 
cross-section of the cord ; but the lateral columns are more 
frequently involved than any other region, and for this rea- 
son the spastic symptoms are, as a rule, the first to be de- 
veloped. In other cases in which the morbid process in- 
vades the posterior columns the symptoms may resemble 
those of tabes rather than those of a spastic form of paral- 



SYPHILIS OF THE SPINAL CORD. 341 

ysis. If the invasion occurs from the ventral surface, as 
it rarely does, the symptoms will naturally be of an 
atrophic order rather than of a spastic or sensory kind. 
But wherever the first point of attack may be, the morbid 
process gradually works its way inward from the periph- 
ery, and its plan of attack is generally from symmetri- 
cally situated points. The cellular proliferation and the 
slight increase in neuroglia tissue, as well as the gradual 
disappearance of the integral elements of the cord, give to 
these cross-sections, if examined under the microscope, the 
appearance of ordinary myelitis, and if it were not for the 
characteristic changes in the blood-vessels and for the evi- 
dence that the process has worked its way inward from the 
periphery, and, indeed, from the pia, the anatomical proof 
of a specific process would be very difficult to establish. 
As it is, there are not a few cases in which satisfactory 
proof cannot be given, and the pathologist is compelled to 
rely upon the clinical evidence in the case to prove the 
syphilitic nature of the disease.* But the wide-spread 
character of the disease, and the very gradual destruction 
of the different systems of the cord, as well as the impor- 
tant part played by the pia and the blood-vessels, leave little 
doubt as regards the true nature of the inflammatory proc- 
ess, and at the same time help us to understand the pecul- 
iar behavior of the clinical symptoms. The Figures 82-84 
will give full details of the specific meningo-myelitis as 
seen in one of my adult cases. 

The meningo-myelitis may be associated with special 
gummatous deposits in any part of the cord or in the brain. 
Thus, in one of my cases a gumma in the pons was asso- 
ciated with a wide-spread specific meningo-myelitis at the 
base of the brain and throughout the entire extent of the 
cord. If such a complication exists the child may present 
the symptoms of tumor of the brain or cord, together with 
the symptoms of extensive meningo-myelitis. 

The fact that spinal syphilis has a distinct tendency to 
improvement makes the prognosis favorable, although this 
tendency implies also a danger of relapses. But syphilis in 

* Unfortunately the bacteriological investigations of Klebs, Disse and Taguchi, 
of Lustgarten, and others, have not yet disclosed the true bacilli of syphilis. 



342 THE NERVOUS DISEASES OF CHILDREN. 

the spinal cord of the adult, as well as of the child, is amen- 
able to treatment, and the prognosis is distinctly more fa- 
vorable than in cases in which the same symptoms might 
be present and not due to syphilis. The prognosis is, for 
instance, very much more favorable than in cases of con- 
genital diplegias or paraplegias. The possibility of recovery 
should therefore be kept in mind, but the hope of complete 
restoration can be entertained only if the symptoms recede 
promptly upon anti-syphilitic treatment. If the symptoms 
do not in any way yield to treatment, and if, after a number 
of careful trials the condition remains practically the same, 
the prognosis is as unfavorable as it would be in any other 
case of myelitis. The prognosis will also depend very 
largely upon the evidence of the amount of damage that 
has been done by the specific process. If the symptoms 
show that there has been a complete destruction in one or 
more systems of the spinal cord the possibilities of recovery 
are naturally less than in cases in which the symptoms point 
to but a slight involvement of these parts. Yet whatever 
the outlook may be at the time the patient is examined, it is 
the physician's duty to warn the parents against the possi- 
bility of relapses and to prepare the relatives for the fact 
that later attacks may involve more vital parts, and may, 
therefore, be more dangerous than the one through which 
the child is passing at the time. 

Treatment. — Syphilis of the spinal cord in the child, as 
well as in the adult, calls for very prompt treatment. We 
must depend upon the usual remedies — the mercurials and 
the iodides. It is wrong to depend upon either one alone, 
for in some cases the mercurials are more effective than the 
iodides, and in others the reverse is true. It is my practice 
invariably to begin treatment with both,* and to stop one or 
the other only if either is not well tolerated or if the im- 
provement is such that I find the patient will do well on 
one drug alone, or if there is no improvement whatever, and 
I feel satisfied that the morbid process cannot be influenced 
either by iodides or mercurial preparations. Mercury 
should be given in the form of inunctions, either of the ten 

* I have never seen any untoward results from the simultaneous use of iodides and 
mercurials in the doses here recommended. Lewin thinks there is danger in this. 



SYPHILIS OF THE SPINAL CORD. 343 

per cent, oleate of mercury or of the unguentum hydrargyri. 
According to the age of the child, one-half to one gramme 
of the ointment may be rubbed in daily, and in addition it 
will be well to begin giving small doses of the saturated so- 
lution of the iodide of sodium, the daily dose to be increased 
slowly. Thus I begin with three or four minims of this 
solution, to be given in milk three times a day, and increase 
the daily dose by one minim until a child, according to its 
age, takes ten, fifteen, or twenty minims of the iodide three 
times a day. As soon as decided improvement takes place 
the quantity of the mercurial inunction given may be re- 
duced and soon stopped altogether; but the iodides should 
be pushed for some time after this improvement has been 
noticed. In cases in which no improvement occurs, in spite 
of the proper administration of these drugs, it is well to 
stop both, for a time at least ; but I would advise, under all 
circumstances, if the diagnosis is safely established, to make 
repeated trials of these drugs in the manner indicated 
above ; and if a child has passed through several attacks of 
specific myelitis it would be well to subject it to periodic 
treatment by the iodides in the same manner that one 
would administer treatment for constitutional syphilis. In 
addition to the anti-syphilitic treatment, the physician will 
have to prescribe tonics, sufficient exercise, and nutritious 
diet, according to the needs of the patient. Moreover, in 
each attack, or as long as the paralysis lasts, it will be neces- 
sary to use electricity and massage, as one prescribes them 
in all other cases of myelitis, whatever the origin of the my- 
elitis may have been. 



BIBLIOGRAPHY. 

Bruce : On Syphilitic Nodose Periarteritis, Edinburgh Medieo-Chirurg. Soc. 

May 2, 1894. Reprint. 
Erb: Neurolog. Centralbl., March 15, 1892. 
Friedmann : Zeitschrift f. Nervenheilkunde, vol. iii.. p. 182. 
Gerhardt : Berliner kl. Wochenschrift, 1893, P- 1209. 
Greiff : Arch. f. Psych., vol. xii. 
Hoffman J. : Neurolog. Centralbl., July 1, 1894. 



344 THE NERVOUS DISEASES OF CHILDREN. 

Hoppe (Cincinnati) : Berl. kl. Wochenschrift, 1893, p. 233. 

Kuh : Zeitschr. f. Nervenhk., vol. iii. 

Lamy: These. Paris, 1893. 

Oppenheim: Report. Berlin, 1890. Berl. kl. W., 1888, 1889. 

Rumpf : Monograph. Wiesbaden, 1887. 

Sachs, B. : New York Medical Journal, Sept. 19, 1891. 

. Brain, vol. xvi., 1893 (contains literature). 

Siemerling : Arch. f. Psychiatrie, vols, xix., xx., xxii. 
Sc human : Arch. f. kl. Med., 1889. 



CHAPTER XIX. 

DISSEMINATED SCLEROSIS. 

Disseminated, insular, or multiple cerebrospinal scle- 
rosis is distinctly a disease of early life. The first pro- 
nounced symptoms appear, generally, in the second and 
third decades of life, but a few prodromata can be traced 
back to a much earlier period.* The disease, as described 
by Charcot, is characterized by a ver}' definite set of symp- 
toms ; but variations from this type are not infrequent. 

Symptoms. — The prodromal symptoms of this disease 
consist of a weakness of the upper, and sometimes of the 
lower, extremities, of a slight awkwardness and trembling 
of the fingers, and of slight subjective sensory disturbances 
in the arms and legs. The chief symptoms develop gradu- 
ally, and are present from the early periods of the disease to 
the very end, but vary much in intensity at different periods 
of the disease. The most characteristic symptom is the 
tremor which is observed on voluntary motion of the af- 
fected parts. It is not observed when the parts are at rest, 
and only when a voluntary effort is made ; it is spoken of as 
" intention tremor." The tremor consists of about six to 
seven oscillations per second (Grasset). It becomes most 

* In a recent dissertation on Multiple Sclerosis in Childhood, by Totzke, it is stated 
that some of the symptoms were manifested in two cases at birth ; in one case at the age of 
five months, in one at fourteen months, and in two at the age of two years ; and of thirty- 
one cases which the same author analyzes, a very fair proportion began between the 
fourth and fourteenth years. He infers from this that the earlier years of life show a par- 
ticular predisposition to this disease ; but there is some reason to doubt whether all these 
cases were typical cases of disseminated sclerosis. In my own practice the youngest 
child that I had occasion to observe with distinct symptoms of multiple sclerosis was 
fourteen years of age at the time of observation, and her symptoms did not come on be- 
fore the tenth year. Marie, who reported three cases of "sclerose en plaques," in 
infants, in 1883, now states that he would not diagnosticate the cases as such, and be- 
lieves the " disease very rare in children.'' 



34^ THE NERVOUS DISEASES OF CHILDREN. 

distinct if the patient is asked to take hold of any object, 
to pass a glass of water to the lips, or to take hold of a 
pencil, as in drawing or writing. At first this tremor is so 
slight that it does not interfere with the execution of the 
simpler movements, but as it increases in intensity it be- 
comes a bar to the use of the hands and legs, and may be 
an annoyance in walking. In attempting to put a glass of 
water to the lips the water is spilt (" the patient's face and 
garments are inundated " — Marie), and in writing the 
tremor is so marked that the pen tears the paper and the 
writing becomes altogether indistinct. 

Next in importance to this tremor is the difficulty in 
speech. The patient speaks deliberately, slowly, pronounc- 
ing each syllable with unusual care (scanning speech), and 
yet a distinct tremulousness of the voice is apparent. The 
consonants 1, p, g, and r, are most difficult to pronounce. 
The words in which these letters occur become unintelligi- 
ble at a very early period of the disease. 

Ocular symptoms are very common ; we may have, at 
times, an inequality of the pupils. (Uhthoff noted this in 
eleven per cent, of his cases.) The reactions to light and 
during accommodation are diminished, and a myosis is gen- 
erally present. Nystagmus appears at a very early day, 
particularly if lateral movement of the eyes is attempted. 
Upward or downward motion is unaccompanied by this 
phenomenon. Visual disturbances are not as common as 
in tabes, but do occur frequently, according to the excel- 
lent studies made of this special symptom by Uhthoff some 
years ago. Clinically we find a narrowing of the field of 
vision, particularly of color, and a diminution of the actual 
visual power. The trouble may be unilateral or bilateral. 
These symptoms are associated with atrophic discoloration 
of the papillae, which persists even though an improvement 
in the subjective symptoms may take place. The eye symp- 
toms are so similar to those observed in hysteria, that on 
this account alone the two diseases have frequently been 
mistaken for one another. 

A distinct tremor of the tongue and a peculiar vague 
expression of the countenance are the other head symptoms 
present in this disease. Charcot described a special facies 






DISSEMINATED SCIEROSIS. 2)47 

of multiple sclerosis, and refers to a vague and uncertain 
look, the lips hanging down and half open. On the whole 
there is an expression of mild stupor on the face ; this ex- 
pression is not altogether unnatural, for the mental condi- 
tion is in many cases abnormal. The memory is weak ; 
there is lack of attention on the part of the patient, who 
passes easily from laughing to crying, and vice versa. Many 
of the patients are emotional to an extreme degree, and this 
is particularly true, according to my own experience, of 
those in whom the disease begins very early in life. 

The paralytic symptoms are of a spastic order. The 
legs and arms are moved awkwardly at first, later with dis- 
tinct evidence of rigidity. A spastic or spastic-paretic gait 
is present, and is associated with those other symptoms 
characteristic of all spastic paralysis and of involvement of 
the lateral columns of the cord, viz., rigidity of the muscles 
and increase of the deep reflexes. All these symptoms 
may for a long time be slight, but as the disease progresses 
they become more and more intense ; the spasticity and 
weakness grow worse until the patient becomes completely 
bed-ridden ; and the tremor increases in intensity and inter- 
feres with the use of the extremities. Speech becomes 
almost unintelligible, the memory grows weaker and 
weaker, until a truly pitiable condition is reached. Ob- 
jective sensory disturbances are not uncommon ; Freund 
has observed them in twenty-nine out of thirty-three cases. 
In fourteen there was a mere transitory anaesthesia, while 
in six cases he states that there was a more or less perma- 
nent and complete loss of sensation. He also refers to the 
occurrence of hyperassthesia, and even to the dissociation 
of sensation, but this latter form of sensory disturbances is 
surely a great rarity. Fortunately, or unfortunately for 
many, the disease covers a long period of years, during 
which a tolerable condition of health is maintained. In 
other cases, again, the symptoms described are developed 
more rapidly, and a few others are added which are not in- 
variably present. This is true of the bulbar symptoms 
leading to difficulties not only of speech but of deglutition. 
and occasionally to interference with the respiratory func- 
tions. 



348 



THE NERVOUS DISEASES OF CHILDREN. 



i\\' 



Paralysis of the ocular muscles, above all of those sup- 
plied by the sixth and third nerves, are not infrequent. Ves- 
ical trouble has been recorded by Erb and Oppenheim. 
If we add to the above symptoms the complete paralysis 
resulting from apoplectiform attacks it will be evident that 

the disease may become almost pro- 
' )$ { \ I , - ; f v tean in its manifestations. Para- 

doxical contractions and impulsive 
laughter are frequent symptoms. 

As important as the positive 
symptoms hitherto recorded are 
those of a negative order. Atrophy 
of the muscles, paralysis of the blad- 
der and rectum, and changes in elec- 
trical reaction are entirely wanting. 
How such a variety of symp- 
toms is possible in this disease will 
be understood easily enough when 
we refer to the morbid anatomy. 
The course of multiple cerebro- 
spinal sclerosis is slow, and though 
invariably leading to a fatal issue, 
death is, in the majority of cases, 
the result of some intercurrent dis- 
ease. 

Etiology. — Disseminated scle- 
rosis comes on most frequently after 
acute infectious diseases, after pow- 
erful emotions, and occasionally af- 
ter traumatic injuries. Oppenheim 
has shown that the disease appears 
after intoxication by metallic poi- 
sons, while Marie believes it to be 
invariably of infectious origin, com- 
ing on after typhoid fever, pneumonia, measles, scarlatina, 
and small-pox. 

Heredity is supposed to play an important role in the eti- 
ology of insular sclerosis. In the broader sense it is true ; 
for multiple sclerosis may indeed affect children who are 
descended from a neurotic stock ; but it is extremely rare 




Fig. 85. — Degeneration of the 
Cauda Equina in Multiple 
Sclerosis. (After Taylor. ) 



DISSEMINATED SCLEROSIS. 



349 



to find the disease in both parent and child. The cases of 
Pelizaeus, quoted approvingly by some authors, are not to 
my mind typical cases of multiple sclerosis ; they belong 
rather to the hereditary form of spastic paralysis. (See 
page 391.) 

Pathological Anatomy. — We can infer from the name 
that the chief lesions in this disease are irregular sclerotic 
patches distributed throughout the greater part of the cen- 
tral nervous system. These patches sometimes occur in the 
brain as well as in the spinal cord ; but there is no rule de- 




FiG. 86. — Sections through the Pons, Medulla, and Spinal Cord, showing Sclerotic 

Patches. (Taylor.) 

termining their first appearance either in one or the other, 
and there is, therefore, as little reason for establishing a 
spinal form or a cerebral form on anatomical as there is on 
clinical grounds. The plaques have, however, a few fa- 
vorite sites ; the white matter of the brain, the pons and 
medulla, the lateral columns in the dorsal and lumbar 
regions are those most favored ; but they also appear in 
the cortex, in the posterior columns of the cord, in the 
cervical region, and even in the cauda equina. (Fig. 85.) 
Charcot was of the opinion that the foci of disease were 
very rare in the cortex of the hemispheres or of the cere- 
bellum ; but Taylor, in a very careful paper on this subject. 
has shown that the patches may be in these parts as well as 
in others. The sclerotic changes may also affect the spinal 



350 



THE NERVOUS DISEASES OF CHILDREN. 



roots and various cranial nerves. The optic chiasm is fre- 
quently diseased. Whether the sclerosis appears in the cen- 
tral nervous system itself, or in the nerves emerging from 
it, the character of the changes is very much the same, the 
main point being that a few, and not necessarily the major- 
ity, of the nerve-fibres are destroyed. 



These patches present 
slightly elevated, at other 
shrunken and contracted. 



^00mir^ , 



a bluish-gray appearance and are sometimes 
times the parts in which they occur appear 
In size they vary from one twenty-fifth to one 
inch. They are, as a rule, 
a little harder than the sur- 
rounding tissue, and Morris 
calls especial attention to 
the nicety with which they 
can generally be easily dif- 
ferentiated from it by the 
naked eye. 

On microscopical ex- 
amination these patches are 
found to consist of a dense 
fibrous tissue, due undoubt- 
edly to the increase of the 
neuroglia and of the con- 
nective tissue. Weigert in- 
sists that the proliferation 
of neuroglia tissue is more 
marked in this disease than 
in any other. In these 
sclerotic portions the my- 
elin sheaths, according to 
the stage of the disease, are 
more or less diseased, but 
the axis-cylinders in the majority of the cases remain normal. The entire 
process may, therefore, be characterized as an interstitial inflammation, and 
it is owing to the preservation of the axis-cylinder that secondary degenera- 
tion in multiple sclerosis is a rare occurrence, indeed; although Buss has 
recorded a descending degeneration in the lumbar cord and an ascending de- 
generation of the columns of Goll, and of the direct cerebellar tract from the 
eighth cervical segment into the medulla oblongata, but his observations are 
surely exceptional. The absence of secondary degeneration also accounts 
for the frequent absence of contractures.* 

* The tremor of multiple sclerosis has been attributed by some to defective isola- 
tion (loss of sheath) of the axis cylinders, on the supposition that the nerve current 
resembles an electric current, and the axis-cylinder an electric wire ; but this is rather 
fanciful pathology. 




Fig. 87. — Vicinity of Locus Cceruleus. 
blood-vessels. (Taylor.) 



r, cells ; v, 



DISSEMINA TED SCLER OSIS. 



351 



Considering the importance which has been attached to the acute infec- 
tious diseases as an etiological factor in multiple sclerosis, it would be nat- 
ural to expect a very early involvement of the blood-vessels in this disease. 
Such an involvement has been found by Ribbert and urged by Marie. Taylor, 
the most recent author on this subject, whom I am willing to follow in this 
matter, because the autopsy in his case was on a young subject, proves that 
in the earlier stages of the disease there is a distinct increase of the smaller 
blood-vessels and capillaries. (Fig. 87.) The specimens examined also ex- 
hibited small hemorrhages and migrating white blood-corpuscles. In some 
of the vessels there was a slight thickening of the walls and an increase in 
the number of the nuclei. The perivascular spaces were dilated. The same 
changes were seen in a second case of Taylor's. In a third case there was 
absolutely no change in the blood-vessels, but in the second and third cases 
the disease had lasted longer, and it is, therefore, doubtful whether similar 
changes in the blood-vessels might not have been present also in these cases 
in the earlier period of the disease. Taylor thinks that we are not justified 
in connecting these vascular changes with the sclerotic process, for the patches 
are not distinctly related to the diseased vessels. Furthermore, vessels are 
often entirely normal in degenerated areas, and in some cases the disease of 
the blood-vessels is wholly wanting, however wide-spread the lesions may 
be. Further autopsies, particularly of cases that have been of short duration, 
are needed to help decide these various points. 



Atypical Forms and Differential Diagnosis. — It 
would be an easy matter to enumerate a large number of 
atypical forms of multiple sclerosis, for the clinical symp- 
toms as developed by Charcot are not distinct in many cases 
which have, however, a direct relation to this disease, repre- 
senting either early forms of the same, or a peculiar local- 
ization of the sclerotic patches. One variety of multiple 
sclerosis with bulbar symptoms bears a close resemblance 
to amyotrophic lateral sclerosis. But in children the com- 
bination of symptoms giving rise to such difficulty in diag- 
nosis is very rare. Then there is the bulbar, or, as Spitzka 
prefers to call it, " the oblongata type " of multiple sclerosis, 
in which the usual symptoms are not nearly as well devel- 
oped as are those pointing to an involvement of the lowest 
cerebral centres. Difficulties in deglutition, in mastication, 
and in articulation as well as phonation, are most promi- 
nent, and for a long time may obscure the usual symptoms 
of the disease. 

A hemiplegic form of multiple sclerosis has also been 
observed in the adult, but I have not yet met with it in 



35 2 THE NERVOUS DISEASES OF CHILDREN. 

children ; nor have I seen an apoplectiform beginning of 
the disease in children, as sometimes happens later in 
life. 

Multiple sclerosis, and forms closely allied to it in children, should not be 
confounded with transverse myelitis. Acute and chronic forms of myelitis in 
children are not rare. The acute symptoms and the inflammatory condition 
upon which they depend disappear promptly enough, but a degeneration is 
set up which leads to the development of spastic and paralytic symptoms. 
These might be taken to be symptoms of multiple cerebro-spinal sclerosis 
unless the history of the patient is carefully considered. The very acute 
onset and the involvement of the bladder, the persistence of sensory symp- 
toms for a shorter or a longer period of time, will help to differentiate myelitis 
and its secondary degeneration from insular sclerosis. 

From congenital spastic paraplegia the disease can be differentiated by a 
consideration of the mode of onset, and by the very early appearance of the 
congenital disease, for insular sclerosis does not generally come on until very 
nearly the first decade of life is passed. Moreover, in the congenital cases of 
spastic paraplegia there is frequently some cerebral defect ; and the cardinal 
symptoms of sclerosis are wanting in these cases. Westphal described a 
pseudo-sclerosis, a condition closely simulating that of multiple sclerosis ; but 
it is not an important disease, for to my knowledge it has not been clearly 
established in any other instance excepting the one reported by Westphal. 

Cases of hereditary tremor might be mistaken for multiple sclerosis, and I 
have myself seen one patient, a girl aged twelve, in whom there was much doubt 
as to whether or not this tremor, which became very much aggravated on 
voluntary motion, was the first symptom of a multiple sclerosis ; but having 
observed the patient for fully five years without the addition of any other 
symptoms referable to a multiple sclerosis, I am confident that the girl is suf- 
fering from an hereditary form of tremor, and have since learned that her 
father and an uncle have suffered from the same disease since very early 
childhood. 

The question also arises in some cases whether an insular sclerosis or an 
hysteria is present, but a consideration of the symptoms will soon remove all 
doubts as to the diagnosis. Nystagmus, scanning speech, and intention 
tremor are exceedingly rare in hysteria, while the sensory disturbances of hys- ' 
teria are too typical to be mistaken for the slight subjective and objective dis- 
turbances of sensation that occur in multiple sclerosis. 

In the adult the chief point in differential diagnosis is 
considered to be that between multiple sclerosis and paraly- 
sis agitans. To this we need pay comparatively little atten- 
tion, since paralysis agitans is distinctly a disease of later 
years, and is rare indeed before the age of forty ; but I can- 
not forbear referring to the case of a patient, still under my 



DISSEMINA TED SCLER OSIS. 



353 



care, who, although but fifteen years of age, presented all 
the symptoms of genuine paralysis agitans. As will be 
seen from the accompanying cut, the position of the body, 
the expression of the face, the position of the hands, are 
typical of paralysis agitans, yet 
the symptoms have changed in 
such a way that at the present 
time they point to a multiple 
sclerosis rather than to a shak- 
ing palsy. (Fig. 88.) 




The patient fell at the age of five 
years, and had an attack of convulsions 
with loss of consciousness, which was 
followed by a slight weakness of the 
legs, but from this condition he rallied 
rapidly enough, and was entirely well, 
exhibiting good physical and mental de- 
velopment. He was bright in school, 
and perfectly well until the age of fifteen, 
when he met with another accident ; he 
fell from a carriage and was badly fright- 
ened. 

The first symptoms he noticed were 
pains around the left ankle, then a shak- 
ing of the left leg. Soon thereafter the 
arm began to shake. Five months later 
he noticed the same symptoms on the 
right side of the body. 

On my first examination of the boy, 
three years after the accident, I found 
him well nourished ; the muscles in good 
condition, the color of the skin normal, 
but considerable vasomotor disturb- 
ances. He exhibited the mask-like ex- 
pression of the face, and his speech, as 
well as the rhythmical tremor of the 
hands, legs, and head, were exactly those 
of a patient suffering from paralysis agi- 
tans. 

Soon another set of symptoms ap- 
peared, which proved to me that this " senile " disease, when occurring in a 
young individual, was subject to peculiar modifications, which allied it much 
more closely to multiple sclerosis, a disease common in earlier life. 

While under observation he developed characteristic nystagmus, scanning. 
23 




Fig. 88.— Patient with Paralysis Agi- 
tans, who also exhibited some Symp- 
toms of Disseminated Sclerosis. 



354 THE NERVOUS DISEASES OF CHILDREN. 

and tremulous speech. All the reflexes became greatly exaggerated. The 
typical tremor- of paralysis agitans, involving the head, lips, and tongue, and 
the extremities, remained unchanged. He still exhibits the propulsive move- 
ment so characteristic of paralysis agitans. The hands have the position of 
shaking palsy, and the contractures are much like those seen in the senile form. 
Motion is almost impossible, and with the symptoms pointing in part to 
multiple sclerosis, and in part to paralysis agitans, he presents a very unusual 
appearance.* 

Prognosis. — Like many other chronic disorders of the 
central nervous system, multiple sclerosis is not a rapidly 
fatal disease. It may run a continuous course for years Avith- 
out seriously endangering life, but any intercurrent disease is 
likely to lead to a fatal termination. At all events, everyone 
will acknowledge that it is rare to see cases of multiple scle- 
rosis that have exceeded the age of forty-five or fifty years, 
and as for those beginning very early in life, the facts are 
altogether too few to warrant us in stating that such pa- 
tients live much beyond the middle period of life. The 
danger to life is greatest in those cases in which the ob- 
longata type predominates. The invasion by disease of the 
vital centres might lead to early death. The invasion is, 
however, a very gradual one, and sudden death need not be 
looked for. In those cases in which such a termination is 
threatened, periods of asphyxia, of aphonia, and other 
symptoms of vagus disturbance would occur, and would 
give warning of the terminal possibilities. 

The disease is absolutely incurable, although remissions 
occur in which all the symptoms excepting the tremor or 
the nystagmus disappear, and in some cases the disease may 
come to a complete standstill for a period of several years. 
In this respect multiple sclerosis is not unlike tabes. My 
notes of the cases beginning in early life would go to show 
that the disease is even more slowly progressive than it is in 
the most favorable adult cases. 

Treatment. — Keeping in mind what was said relative 
to the prognosis of the disease little can be expected from 
our attempts at treatment. But if we cannot cure the dis- 
ease we can at least secure greater comfort for the patient. 
As the tremor is most marked when the patient is ac- 

* Schultze has reported a similar case in Virchow's Archiv, Bd. LXVIII. 



DISSEMINATED SCLEROSIS. 355 

tive — and the disease is exhausting at best — it is wise to 
keep the patient in bed for a prolonged period of time. The 
rest cure I have found as efficient in these chronic organic 
diseases of the central nervous system as I have in the treat- 
ment of purely functional troubles. It is often surprising 
to note how much benefit, and, above all, how much comfort, 
patients derive from a complete rest when they have been 
attempting to go about from place to place, often from phy- 
sician to physician, with their weak and shaking limbs. 

With this rest treatment I am in the habit of com- 
bining mild hydro -therapeutic procedures. Generally a 
tepid bath in the morning, followed by lukewarm and 
successively colder douches down the spine, or by the 
use of the drip-sheet. At night it is well to give a tepid 
bath without douches, as the former contributes much 
to the sleep and the general contentment of the patient. 
The galvanic current may be employed to advantage. 
Stabile currents of 10 to 15 milliamperes may be passed 
down the spine and through the extremities. The faradic 
current should, however, be avoided, as it tends to increase 
the spastic rigidity of the muscles. Massage is also in or- 
der in these cases, if intelligently adimnistered. Spastic 
rigidities yield a little to such manipulations, and accord- 
ing to the recent investigations of Mitchell and his son, the 
general condition of the patient is improved by massage. 
If treatment is directed in this way and adapted to each 
individual case it will be far more satisfactory than send- 
ing the patient away to various springs, where a general 
routine treatment is adopted and the patient subjected to 
it, whether he suffers from rheumatism, gout, syphilis, or 
what not. 

Various drugs have been employed and recommended in 
cases of multiple sclerosis. Among these nitrate of silver, 
the iodides, and mercurials are the most prominent. I am 
confident that the first never does any good excepting pos- 
sibly to allay pain, and the last two will not be needed, for 
it is very certain that multiple sclerosis is rarely developed 
on a syphilitic basis, and there is no indication for the use 
of resorbents. 



356 THE NERVOUS DISEASES OF CHILDREN. 



BIBLIOGRAPHY. 

Charcot: Lecons, etc., vol. i., p. 399, 1892; On Visual Troubles, Semaine 

Med., February 14, 1891. 
Freund : Arch. f. Psych., etc., 1891, vol. xx. 
Grassetet Rauzier : vol. i., p. 796. 
Kahler u. Pick : Prager Vierteljahrsschrift, 1879. 
Marie : Maladies de la Moelle, p. 108. 
Mensi : Riforma Medica, November, 1892. 
Nolda : Arch, fur Psych., vol. xxiii. 
Oppenheim : Berl. kl. Wochenschrift, 1888. 
Pritchard : Keating's Cyclopaedia. 
Ribbert : Virchow's Archiv, vol. xc. 
Sachs : New York Medical Journal, September 1, 1894. 
Schultze : Virch. Arch., vol. lxviii., p. 120. 

Spitzka : Article on Scleroses of Spinal Cord, in Pepper's System, vol. v. 
Taylor : Zeitschr. f. Nervenheilk., vol. v. 
Uhthoff : Arch. f. Psych., vol. xxi. 
Westphal : Arch. f. Psych., vol. ix. 



CHAPTER XX. 

COMPRESSION OF THE SPINAL CORD— POTT'S PARALYSIS. 

Injury to the cord may be due to specific diseases in 
the bone, to compression by an aneurism or to growths orig- 
inating in the membranes. These conditions are very rare, 
however, in children, and whenever the symptoms point to 
compression of the cord, caries of the bone may be taken to 
be the primary trouble. Pott's disease of the spinal column 
begins so insidiously that the recognition of it and of the 
symptoms resulting from compression are reached frequent- 
ly by exclusion only. While the disease may develop at 
any period, it is more common in childhood and in early 
life ; it is the result of a tubercular or scrofulous diathesis ; 
and evidence is easily adduced in the majority of cases of 
the existence of such diathesis in the same family and in the 
same or in preceding generations. Slight traumatic injuries 
are often sufficient to bring about marked disease of the 
bone, but evidently only in those children who are predis- 
posed to such disease, so that here as elsewhere the trau- 
matic incident simply helps to bring out the latent tendency 
to disease. There is often a period of weeks or months 
between the time of injury and the first symptoms of the 
disease. In consequence of the caries of the bone, the bodies 
of the vertebras become softened and are displaced, two or 
three of them, or even more, forming a marked angular 
curvature. Such angular curvature may in rare cases be 
the result of a septic process, in which case an abscess may 
have formed in the bone and caused the softening. Gen- 
erally the bodies of the vertebrae become diseased and the 
intervertebral substance undergoes inflammatory changes 
as well. The pressure upon the softened parts is sufficient 
to account for the deformity. 



358 THE NERVOUS DISEASES OF CHILDREN. 

The deformity resulting from the disease of the bone is 
of such a character that a sharp angle is usually formed at 
one point, at which the cord is generally much compressed. 
In some instances the cord becomes quite thread-like. It is 
remarkable that the cord may sustain a very large amount 
of compression before its functions are seriously interfered 
with, or the symptoms of disturbed function appear. This 
accounts for the fact that in many instances the angular 
curvature may be extreme and yet neither paralysis nor 
anaesthesia is observed during life. Lateral displacement 
may occur without its having any influence upon the cord. 
Very naturally the spinal cord suffers under these condi- 
tions, and the roots emerging or entering through the in- 
tervertebral foramina are compressed by the thickened 
sheaths or by other inflammatory products. This accounts 
for the frequent occurrence of pain in these cases, and for the 
occurrence of paralysis in cases in which the cord itself 
shows tolerably normal structure. 

The causes of injury to the spinal cord in the subjects of 
Pott's disease are twofold : first, the cord is actually com- 
pressed in many cases, and, secondly, it suffers from the in- 
flammatory products gathering between the cord and the 
bony parts. The latter cause is often quite as active as the 
former. The changes in the cord itself will vary very 
largely according to the degree of compression. The cord 
is somewhat altered, the normal white having given way to 
a grayish or reddish-gray tint. The cord is less hard than 
under normal conditions, both these changes being clearly 
due to inflammatory processes. It has been found by 
Schmauss and others that the amount of inflammation of 
the cord is not always in keeping with the degree of com- 
pression. Recent French authors have attached consider- 
able importance to an oedema of the cord produced by 
compression of the veins as they issue from the cord. The 
oedema is subject to fluctuations ; and a diminution of the 
same may be responsible for an improvement in the pa- 
ralysis. 

The symptoms of Pott's paralysis often set in long be- 
fore there is any possibility of actual compression. In those 
cases we must suppose that the symptoms are due to a 



COMPRESSION- OF THE SPINAL CORD. 359 

pachymeningitis constricting the spinal roots and blood- 
vessels ; the occurrence of rapidly developing spinal abscess, 
associated with painful paralysis, and the disappearance of 
the paralysis as soon as the abscess is emptied, lend support 
to this view. By degrees an extension of the inflammatory 
process takes place into the substance of the cord. The. 
myelitis as it occurs Avith Pott's disease shows a general in- 
crease in the interstitial tissue, with proliferation of the 
various cells and degenerative changes in the more impor- 
tant nerve elements. Granular corpuscles, corpora amylacea, 
and globular masses of myelin are found in sections that are 
properly hardened. The gray matter may have become 
almost indistinguishable from the white, and the larger 
ganglion cells may have entirely disappeared from the for- 
mer. In addition to these changes the usual ascending and 
descending degenerations will occur, as from any other 
focus of inflammation in the cord. The changes in the pe- 
ripheral nerve-roots will be those that would be expected 
from actual compression and inflammation. The interstitial 
tissue will be increased, the nerve-fibres themselves will be 
wasted, and the axis-cylinders will show the usual changes 
of degeneration. 

Symptoms. — The chief symptoms in this disease are a 
very gradual development of a spastic paralysis, generally 
of the legs and possibly of arms and legs ; the reflexes are 
increased, and radiating pains are present at a very early 
period of the disease. The symptoms often resemble those 
of a dorsal myelitis, since Pott's disease attacks the dorsal 
region more frequently than any other. If other regions 
of the spine are diseased the symptoms will vary as the)' 
would in the various forms of myelitis, but root symptoms 
will always play a very prominent part. The symptoms 
due to compression of the root - fibres will also help to 
differentiate between a myelitis following Pott's disease 
and the ordinary traumatic or non-traumatic myelitis. The 
compression of the sensory nerve-roots causes seyere pains. 
It may also interfere with normal sensation, and if both 
these functions are disturbed, an anaesthesia dolorosa may 
be well developed and associated with the paralysis. Be- 
fore the condition of anaesthesia is established a transitory 



360 THE NERVOUS DISEASES OF CHILDREN. 

hyperesthesia may have existed. The irritation of the 
motor root-fibres would lead us to expect a condition of 
involuntary contractions of the muscles, but, as a matter of 
fact, these are not so frequent, and probably because the 
ventral gray matter of the cord, being nearest the verte- 
bral bodies, is affected at an early day ; and since the func- 
tion of this gray matter is impaired, irritation of the an- 
terior roots connected with it will not be able to exhibit 
symptoms of irritation. According to the location of the 
disease in the bone the symptoms may for a time remain 
unilateral; but, as a rule, the disease spreads rapidly 
enough to bring about a bilateral, and often symmetrical, 
set of symptoms within a very short period of time. Fur- 
thermore, as the disease progresses the paralyzed parts may 
become atrophied, the vesical and rectal reflexes may be 
impaired, and we then have all the ordinary symptoms 
of myelitis, even including bed-sores and the usual trophic 
disturbances of the skin. 

The course of the disease may be inferred from the ra- 
pidity with which all these symptoms are developed. If 
the disease is of a slowly progressive character, the symp- 
toms will naturally be developed one by one, whereas if the 
bone disturbance is of a more pernicious and rapidly pro- 
gressive character the symptoms of a complete myelitis 
will be reached at a very early day.* 

Diagnosis. — Paralysis due to Pott's disease must be 
distinguished from subacute or chronic myelitis. This dis- 
tinction is not always an easy one to make. It is reached 
most readily in those cases in which the course of the dis- 
ease is a very slow one and the usual causes leading to a 
transverse myelitis are wanting. It is rendered probable 
by the coexistence of pain with the paralysis, and above all 
things by the discovery of tenderness to pressure over the 
spinous processes. These same symptoms might occur in 
cases of tumor of the cord, but the unilateral development 
of symptoms in the latter disease will soon lead to a safe 
differential diagnosis. (See next chapter.) The earlier 
symptoms of a Pott's paralysis may simulate the condition 

* In some cases, like the one reported by Daxenberger, the caries may be latent for 
years, before spinal-cord symptoms are developed. 



COMPRESSION OF THE SPINAL CORD. 36 1 

of spastic paraplegia, either of the congenital order or of 
that form due to specific disease. 

In several cases, under the author's care, the symptoms of Pott's paral- 
ysis were developed long before any actual evidence of disease of the verte- 
brae could be elicited. Whitman also states that in a number of cases the 
paralysis precedes the deformity. The diagnosis must at times be reached 
by exclusion ; but the condition may be suspected in cases of a gradually de- 
veloping spastic and painful paralysis, in which there is no trace of traumatic 
injury, and no history of any acute infectious disease immediately preceding 
the onset of the symptoms. 

The age of the patient and his general appearance will 
also help to establish a differential diagnosis. 

Prognosis. — The progress of the paralysis will depend 
mainly upon the development of the disease in the bone. 
If the disease has lasted long enough to produce complete 
compression at one point, the spinal-cord symptoms may per- 
sist during life, although the bone disease be checked or re- 
covered from. In other cases the course of the spinal-cord 
disease may be checked, although the disease of the bone 
steadily progresses. With the subsidence of the inflamma- 
tion the sensory symptoms may diminish, but the condition 
of paraplegia may continue years thereafter as a natural re- 
sult of the changes that have been set up in the spinal cord. 
In a number of cases, however, there is a proportionate de- 
velopment between the disease of the bone and the disease in 
the spinal cord. In the more severe cases the paralysis re- 
mains unaltered, or is steadily progressive, and all those 
complications arise, such as bed-sores, cystitis, and the like, 
which help to bring about a fatal issue in many cases of my- 
elitis. In the milder cases recovery is to be expected, and 
many a child who has not been able to walk for years during 
the active period of caries has attained a tolerably normal 
gait in later life. On the whole, the motor symptoms are 
much more persistent than the sensory. The prognosis is 
much more grave in the cases of cervical Pott's disease than 
in those occurring in the lower vertebral reo-ions, for rea- 
sons which can be readily understood. 

Treatment. — In the treatment of paralysis resulting 
from Pott's disease surgical methods should be resorted to 



362 THE NERVOUS DISEASES OF CHILDREN. 

at the very beginning. If the spinal cord is to recover, the 
tubercular process in the spinal column must be checked 
first of all. The old method of absolute rest should be em- 
ployed, and tonics, such as cod-liver oil and iron, should be 
given. I do not wish to enter into the discussion as to which 
of these measures, the rest or the tonics, is more impor- 
tant. Some will be cured by the one, and some by the other, 
method, but I have not the slightest doubt that if all these 
measures are employed conjointly the result will be more 
satisfactory. In addition to rest, absolute immobility should 
be secured, either by forcible extension in bed, or by the 
application of a plaster-of- Paris jacket. The plaster-of- 
Paris jacket is not so useful during the period of rest as it is 
during the period of partial recovery, in which it is desira- 
ble for many reasons that the child should be taken out of 
bed and enabled to walk in the open air. I am very certain 
that the application of counter-irritants, which is still recom- 
mended by many, is of no use, and simply helps to increase 
the tortures that these children suffer. Of late years active 
surgical interference has been suggested in these cases by 
Macewen, Horsley, and others, but the wisdom of this may 
still be doubted. The surgeon's skill should, at all events, 
be restricted to those cases in which the clinical symptoms 
prove that there is evidence of compression at any one spe- 
cial point by inflammatory products or by displaced bone. 
If the child is strong enough to tolerate such surgical pro- 
cedures, the removal of these products of inflammation 
may be attempted ; but it is well to caution the physician 
who may be eager to try new surgical procedures that chil- 
dren are less tolerant to spinal and cerebral surgery than 
adults. 

After surgical measures have been given a fair trial, the 
physician and neurologist will be called upon to remedy the 
paralytic condition. Some assistance can be given by the 
orthopedic surgeon, who, by the ordinary tenotomies, may 
enable the child to use its feet to greater advantage. His 
treatment will be supported in every case by the discreet 
use of electricity and massage. The ordinary blood and 
nerve tonics will help very much more than the iodides 
or mercurials, but these should be given in the earliest 






COMPRESSION OF THE SPINAL CORD. 363 

stages of the trouble. I have seen a favorable result from 
the latter in one case in which the process was suspected 
to be gummatous and not tubercular. 



BIBLIOGRAPHY. 

Charcot: Lecons, etc., 1873. 

Daxenberger : Zeitschr. f. Nervenheilkunde, vol, iv., p. 136. 

Erb ; Ziemssen's Cyclopaedia, vol. xi. (literature to 1878). 

Grasset et Estor : Revue de Medecine, Feb., 1887. 

Kahler: Zeitschr. f. Heilkunde. Prag, 1882. 

Kahler u. Pick : Arch. f. Psychiatrie, vol. x. 

Leyden : Klinik der Rickenmarkskr., vol. ii., p. 151. 

Schmaus : Monograph. Wiesbaden, 1890. (Contains a very full summary 

of cases with autopsies.) 
Whitman : Differential Diagnosis of Pott's Disease in Childhood, New York 

Polyclinic, May, 1893. 



CHAPTER XXI. 

TUMORS OF THE SPINAL CORD AND ITS MENINGES. 

The study of tumors of the spinal cord was at one time 
consdiered to be of small practical value on account of the 
rarity of the affection ; but far greater interest attaches 
to it now from the fact that the removal of these tumors 
has been rendered possible by the recent advances in sur- 
gery. A few years ago Mills and Lloyd collected and 
analyzed fifty cases of cord tumors ; fourteen per cent, of 
these were under twenty years of age. Of these four oc- 
curred before the age of ten and three between ten and 
twenty. 

Causes. — The actual cause of tumors of the spinal cord can be deter- 
mined as little as in the case of cerebral tumors. They are rarely primary, 
and are commonly due to some constitutional diathesis, such as tuberculosis 
or syphilis, or are the metastatic products of malignant tumors in other organs 
of the body. The tendency to proliferation of nerve-tissue is not so marked 
in the spinal cord as in the brain of the child. Gliomata originate in the vicinity 
of the central canal, and here are of special interest, as they often break down 
and lead to the formation of cavities, and hence have given rise to the discus- 
sion whether these cavities are invariably the result of such disorganization 
of a neoplasm, or whether the condition of syringo-myelia may be the result 
of congenital enlargement of the central canal. Small hemorrhages and em- 
bolism, though much rarer than in the brain, may give rise to cystic forma- 
tions, but among cystic growths those due to parasitic infection, particularly 
to the echinococcus, are the most common. As in the case of brain tumor, 
traumatic injury has been declared responsible by many for the occurrence of 
spinal tumors. It is doubtful, however, whether traumatism plays any further 
role than that either of eliciting a latent tendency to such disease, or of 
merely calling attention to the symptoms of the disease which may have 
antedated the injury. 

Symptoms. — The symptoms of tumor of the cord might 
be deduced from a consideration of the disturbance of 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 365 

function due to a very gradual invasion of the cord. Thus 
in many cases the symptoms are strictly unilateral for a 
time, and become bilateral with the extension of the disease 
from one part of the cord to the other. The symptoms will 
naturally vary also according to the seat of the disease, ac- 
cording to the more or less rapid destruction of the cord, 
and according to the development of the tumor in the 
meninges or in the spinal cord itself. It is of the greatest 
importance to distinguish between extra-medullary and me- 
dullary tumors. Extra-medullary tumors are characterized 
by the early appearance of root symptoms, more persistent 
pain in the back, and by sensory disturbances which point 
to compression of the posterior root-fibres ; by paralysis 
and atrophy if the growth is on the ventral surface. These 
distinctions will not hold good if, as in a case of my own, 
the intra-medullary tumor starts near the entrance of the 
posterior root-fibres and actually compresses these. In 
some cases, however, the disproportion between the sen- 
sory symptoms and the paralysis will direct attention to 
the seat of the tumor outside of the spinal cord, particu- 
larly if all symptoms pointing to involvement of the cen- 
tral gray matter are absent. The focal symptoms in ex- 
tra medullary, as well as in intra-medullary tumors will 
vary greatly according to the seat of the tumor. There 
may be but slight sensory disturbances in a single area 
or in one limb; the sensory disturbances may be uni- 
lateral, and these may or may not be associated with par- 
tial paralysis, either unilateral or bilateral ; or there may be 
crossed sensory and motor symptoms. Any set of spinal, 
and more particularly hemi-spinal symptoms, whether of a 
motor, sensory, or vasomotor order, if they be slowly pro- 
gressive, are suspicious of tumor. There will be additional 
reason to suspect tumor if there is evidence of a distinct 
constitutional diathesis. All the symptoms of various 
spinal-cord affections may be simulated by tumor, accord- 
ing to the part of the spinal cord affected, and in this 
respect the symptomatology of tumors may be quite as 
varied as that of syphilis of the spinal cord. 

If we endeavor to analyze the symptoms in detail, we 
find that disturbances of sensation are by far the most com- 



366 THE NERVOUS DISEASES OF CHILDREN. 

mon. If the tumor is in the cervical region, the pain radi- 
ates into the upper extremities, and generally into definite 
parts of the brachial plexus ; if in the dorsal region, the 
pain is relegated to the region of the ribs, the thorax, or the 
abdomen ; if in the lumbar region, the pain is described as 
limited to the various parts of the lumbar or sacral plexus. 
Whatever the level of the lesion may be, the sensory dis- 
turbances may be unilateral for a time and gradually be- 
come bilateral. Vasomotor disturbances occur, similar to 
those in peripheral neuritis ; the skin becomes glossy, livid, 
and painful. With these symptoms muscular wasting and 
paralysis will soon be associated. As the disease progresses 
the hyperalgesia or the paresthesia gives way to complete 
loss of sensation, the paralysis becomes complete, and the 
vasomotor disturbances increase, and serious trophic dis- 
turbances may set in. The sensory changes may vary in 
still another respect. Since the tumor need not invade all 
sensory fibres at once, some may remain entirely intact, 
while the function of others becomes seriously impaired. 
Thus dissociated sensation may be present in some cases, 
but is rarely as distinctly developed as in the case of syringo- 
myelia, which, as we have seen above, may or may not be 
connected with the formation of a central neoplasm. Ac- 
cording to well-known physiological laws, and to the old- 
time teachings of Brown-Sequard, one would expect in 
many of these cases of tumor to find distinct symptoms of 
unilateral lesion of the cord, namely, paralysis in one mem- 
ber or in one-half of the body, with loss of sensation in the 
opposite half ; but this, curiously enough, is not often the 
case. 

The paralysis will be developed in accordance with the 
ordinary laws relating to spinal-cord affections. The mus- 
cles represented in the segment or segments involved by 
the neoplasm (see tables in Chapter I.) will not only be 
paralyzed, but will also undergo atrophy, while the mus- 
cles represented in lower segments will show spastic forms 
of paralysis without any atrophy. The changes in electri- 
cal reactions will also vary in accordance Avith these con- 
ditions, the only muscles exhibiting distinct reaction of 
degeneration being those governed by cells in the affected 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 367 

segments. Spasmodic contractions, the ordinary rigidities 
and contractures, will be observed in these cases under the 
same conditions as in cases of myelitis. All the symptoms 
will be largely influenced by the fact that an active my- 
elitic process is started up sooner or later in the vicinity of 
most of the tumors. This is particularly true of tubercu- 
lar and gummatous deposits, whereas other forms of tumor 
may exist for some period of time without seriously affect- 
ing the tissue in their vicinity. 

Paralysis of the sphincters of the bladder and rectum 
will occur in cases of tumor as in cases of myelitis. In the 
case of tumor in the lumbar region, absolute paralysis of 
the sphincters and retention are the natural results, while 
if the tumor is above this level incontinence will be the 
more frequent condition ; but in the later stages retention 
with overflow is the rule. The conditions of the sphincter 
ani will vary in very much the same way. As tumors are 
more frequent in the cervical and dorsal regions, I propose 
giving the condensed history of a case, which I had occa- 
sion to observe some years ago, of cervical tumor of the 
cord, although this occurred in an adult and not in a child, 
but the age of the person would have no effect upon the 
character of the symptoms. 



The patient's symptoms were, pains and weakness in his left arm, in- 
ability to move the fingers, and glossy appearance of the left hand. There 
was no history of syphilis nor of tuberculosis in the family. Four weeks 
before I saw the patient he felt what he termed rheumatic pains in the left 
shoulder ; these moved down to the left arm. The hand grew weaker after 
the lapse of a week or more, and then the fingers became puffy and the skin 
glossy. The examination revealed the following conditions : The left arm 
could not be raised as readily as the right ; the fingers were slightly flexed, 
and voluntary extension or further flexion was impossible. There was 
hyperassthesia of the entire forearm and hand, most marked over the distri- 
bution of the ulnar nerve, and particularly over the outer dorsal surface. 
The mere touch with the finger was painful. A prick was so disagreeable 
that he could not bear to be touched with an cesthesiometer. Cold water 
dropped on the arm produced excessive pain. The skin was swollen be- 
tween the joints of all the fingers of the left hand, and there was a peculiar 
cedematous appearance of the skin of the entire hand, including the fingers. 
The electrical conditions were normal. There was no ataxia in the lower 
extremities ; no inco-ordination of movements in the right arm. All super- 



368 THE NERVOUS DISEASES OF CHILDREN. 

ficial reflexes were gone except a very slight scrotal reflex on the right side. 
At this examination the only other symptoms were a slight dragging of the 
left foot, an increase of both knee-jerks, and absence of ankle clonus on 
both sides. Passing over the change in the symptoms during the first few 
weeks, I will merely state that the paresis in the left leg gradually increased. 
There was continued hyperesthesia of the whole leg for several weeks, and a 
gradual weakening of the right leg as well. The abdominal muscles also 
were weakened so that he could not press properly when going to stool. 
Incontinence of urine gradually developed. For some time there was no 
disturbance of sensation in the right half of the body. About one month 
after the first examination the patient began to exhibit a slight cough, and 
was annoyed by the lack of sufficient muscular power to expectorate freely, 
but the physical examination revealed no distinct signs of disease excepting 
a slight catarrhal condition. The paralysis increased rapidly, so that the 
patient was unable to leave his bed. There was also some tenderness over 
the seventh cervical vertebra. The right hand gradually grew weaker and 
sensory disturbances appeared in the right forearm, but his right hand did 
not show the trophic disturbances so prominent in the left. The hyperses- 
thesia which had existed at the beginning gradually gave way to an increas- 
ing anaesthesia. The anaesthesia, six weeks after the first examination, in- 
volved both sides of the body, the only parts retaining normal sensation 
being the neck, face, and head The various forms of sensation were very 
much disturbed. Heat could not be appreciated, but cold was painful for a 
time. The muscular sense was impaired both in the left and in the right leg, 
but in the right arm muscular sense was not disturbed. The only further 
changes noted were an increase in the paralytic symptoms in the left upper 
extremity, though the patient retained considerable power in the pectoralis, the 
deltoids, biceps, and brachialis during the entire disease. The temperature 
was only little above normal. The pulse was small and accelerated, often 
about 1 20. Respiration varied very much, generally about 25. The urine 
was free from sugar and albumin. There was no history of night-sweats, 
but the patient stated of his own accord that he was frequently subject to 
unilateral sweating of the right side. The patient died very suddenly, 
exactly six weeks after my first examination. The autopsy revealed a round 
tumor of the size of a hazel-nut between the sixth and seventh cervical seg- 
ments, pressing closely upon the emerging posterior fibres without displac- 
ing these and extending inward as far as the median line. At its caudal end 
the tumor had exceeded the median line by a small fraction of an inch. 
The tumor proved to be a typical solitary tubercle. In addition to this we 
found that the cord between the seventh cervical and the fourth dorsal seg- 
ments was practically a diffluent mass revealing no trace of structure. 
There were also minute tubercular deposits discovered in both lungs, and in 
both apices there were incipient cavernous spaces. 

The variation of symptoms in other cases would depend solely upon the 
different site of a tumor, but the general course and variability of the symp- 
toms are well illustrated by the above case. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 369 

Pathology. — The morbid anatomy of tumors of the 
cord differs very little from that of tumors of the brain. 
The central nervous system is in this respect a unit, and the 
same growths which are apt to occur in the brain occur, 
though with lesser frequency, in the spinal cord. In the 
cases of Mills and Lloyd, only four were of a tubercular 
character, while twelve were either sarcomata or gliomata. 
Parasitic growths occurred in three cases of the fifty, syph- 
ilitic growths in five, and the remainder were distributed 
among various kinds of neoplasms, such as myxomata, fibro- 
mata, carcinomata, and so forth. Neuromata of the cauda 
equina must be included in the list. The largest collection 
of cases of solitary tubercle of the spinal cord has been 
made and carefully analyzed by Dr. Herter. His experi- 
ence proves that the disease is one of early life, twenty of 
the twenty-six cases occurring before the age of thirty-five. 
A few authors have reported cases of tubercle which they 
supposed to be primary ; but, as in the case reported by 
myself, although it was difficult to prove any other focus of 
tubercular disease during life, such focus of disease evi- 
dently precedes the spinal disease in every instance. Spe- 
cific infiltration of the spinal cord is, on the whole, more fre- 
quent than the same infiltration of the brain. To be sure, 
relatively few cases of this description are on record, but I 
am certain that more careful examination will hereafter re- 
veal a larger number of gummatous deposits, if not gum- 
matous growths, in different parts of the cord, associated 
with the specific meningo-myelitis, which is, as has been 
stated in another chapter, the most frequent form of syphi- 
litic disease of the cord. Tumors of the cord may occa- 
sionally erode the surrounding bony parts and may work 
their way to the surface, but this is very much rarer indeed 
than a similar invasion of the skull in cases of brain tumor. 
The reverse course, from the bone to the cord, may also 
occur, but is a great rarity. 

Diagnosis. — Tumors of the spinal cord are to be differentiated from 
hemorrhage into the cord, from spinal caries with Pott's paralysis, from 
pachymeningitis cervicalis, transverse myelitis, syringomyelia, neuritis, and 
from traumatic injuries of the cord. 

In the case of hemorrhage the paralysis and other symptoms resulting 
24 



370 THE NERVOUS DISEASES OF CHILDREN. 

therefrom appear with great suddeness. The condition is one of extreme 
rarity, and in many of the cases leads to complete paralysis, with all its com- 
plications, and to death in a relatively short period of time. A confusion can, 
therefore, exist only in the first few days of the disease, and under such cir- 
cumstances the suddenness of the onset would argue strongly in favor of 
hemorrhage. 

Spinal caries, with the resulting paralysis, may occasionally present symp- 
toms similar to those of spinal tumor. In both, pressure symptoms are pres- 
ent, but in the case of caries of the bone the symptoms are not nearly so 
intense, as a rule, as in those due to the presence of neoplasm. The chief 
reliance in differential diagnosis must, however, be placed upon the detection 
of bone trouble in the case of spinal caries, although, as we have seen, such 
evidence does not always precede the onset of pressure symptoms. The 
more rapid course of the symptoms in spinal tumor, and the negative results 
of treatment, will also help to differentiate the two conditions. In caries of 
the vertebras, symptoms pointing to the ventral (anterior) surface are a little 
more frequent than in tumor. 

As between pachymengitis cervicalis and tumor of the cervical region, there 
are but few points of differential diagnosis, and the diagnosis will have to 
rest very largely upon the excessive rigidity, the very typical distribution of 
the paralysis in the hand, and the more acute pain in cases of pachymenin- 
gitis than in those of tumor. In tumor, moreover, the symptoms are more 
apt to be unilateral for a time, while those of pachymeningitis are, as a rule, 
bilateral and symmetrical. 

Some of these same points of differential diagnosis will help us also to 
distinguish between transverse myelitis and tumor of the cord, the chief 
points of difference being these : In myelitis the onset is much more rapid 
than in tumor, there is less pain, and the entire cross-section of the cord 
shows symptoms of involvement very much more promptly in myelitis than 
in the majority of cases of neoplasm. A doubt as to which of these two 
conditions is present can exist in the first few days or weeks only ; after this 
the general course of the symptoms will determine the character of the morbid 
process. 

Syringo-myelia is closely related to tumor of the cord ; there is doubt, in 
fact, whether some, if not many, of the cases of syringo-myelia do not repre- 
sent primary neoplasm of the cord, with secondary enlargement of the central 
canal and a breaking down of the spinal tissues. But the gliosis which may 
or may not be the starting-point of a syringo-myelia, takes an extremely 
chronic course, and it is, therefore, by this fact alone, as well as by others, 
that the two diseases can be sufficiently distinguished from one another. In 
syringo-myelia the symptoms are often strictly bilateral, or symmetrical, and 
the disturbances of sensation (the loss of pain and temperature sense, while 
the tactile and muscular sense are preserved) are very typical of syringo-my- 
elia and are rarely met with in cases of spinal tumor. In syringo-myelia 
there is also evidence in many cases of a slowly ascending or descending 
morbid process and such slow ascent or descent is not common in cases of 
tumor. The differential diagnosis is one of great importance, for the one 






TUMORS OF THE SPINAL CORD AND ITS MENINGES. 37 1 

disease is compatible with prolongation of life, while the other is rapidly de- 
structive. (See end of this chapter.) 

The symptoms of neuritis may for a time obscure those due to tumor. 
In cases which I have observed the first symptoms that appeared were those 
of a neuritis involving various branches of the brachial plexus. These were 
due to compression of the spinal roots, and are naturally present in those 
cases in which the tumor begins either in the meninges or near the surface 
of the cord. The early appearance of complete paralysis and the develop- 
ment of other symptoms, particularly those pointing to interference with the 
vesical and rectal reflexes, will argue in favor of neoplasm and not of a sim- 
ple neuritis. The rapid progress of the symptoms in the case of tumor, the 
entire absence of fever in most of them, will also point to tumor rather than 
to neuritis. The presence of sensory and paralytic symptoms in both halves 
of the body, or in the lower as well as upper extremities, without involvement 
of the bladder or rectum, may be considered as pointing to a multiple neuritis 
rather than to tumor. 

A localized specific meningitis may at times simulate tumors of the cord, 
and, as was indicated before, the frequent association of gummatous de- 
posits with such specific meningo-myelitis may present unusual difficulties of 
differential diagnosis. In one case under my observation for a period of 
several months, it was impossible to differentiate accurately between these 
two conditions ; and unfortunately the result of treatment is not to be de- 
pended upon, for even in cases of a distinctly specific character the symptoms 
do not always yield to antisyphilitic treatment. 

Traumatic injuries of the cord may present symptoms resembling those 
of tumor. But the history of the case, and the extreme painfulness over 
the spinous processes, will prevent the possibility of confusion between the 
two. y 

Prognosis. — In spite of some few surgical successes 
tumor of the cord is an extremely grave disease. Spinal 
growths are secondary to malignant disease in other parts 
of the organism and help to intensify the gravity of the dis- 
ease elsewhere. In the case of tubercle, sarcomata, and car- 
cinomata the prognosis is absolutely bad, while it is only a 
little better in cases of gummata. 

The duration of the disease varies according to the site 
of the tumor and according to the morbid character of the 
new growth. Tumors of the cervical region are, on the 
whole, more rapidly fatal than those in the dorsal or lumbar 
region. Tubercles, carcinomata, and sarcomata, take a more 
rapid course, as a rule, than the other forms of neoplasm. 

Treatment. — Tumors of the spinal cord and its me- 
ninges cannot be sensibly affected bv drugs. There is a 



372 THE NERVOUS DISEASES QF CHILDREN. 

possible exception in the case of gummata ; yet, as was 
intimated above, the effect of specific treatment in these 
cases is, to say the least, extremely doubtful. I would ad- 
vise, however, that every case of tumor of the cord be 
given the benefit of the doubt, and that active specific 
treatment by iodides and mercurial inunctions be attempted. 
If these drugs avail little, no other drug need be exhibited. 

Much has been expected of surgical treatment ; but, as 
in the case of cerebral tumors, our hopes in this direction 
have not been realized. Extra-medullary growths are the 
only ones that we can expect the surgeon to remove. Suc- 
cessful cases of this description have been reported by 
Gowers, Horsley, Laqueur, and others.* The cases most 
favorable for operation are those which occur near the sur- 
face of the cord or in the meninges, and those in which the 
operation is done at a relatively early day, before the neo- 
plasm has invaded the cord or has been followed by sec- 
ondary myelitis and secondary degenerations. The case 
published by Laqueur, in which the tumor was found out- 
side of the dura mater and compressing the cauda equina, 
was an exceptionally fortunate one, for the tumor was 
easily found and the effects of compression of the cord were 
not very marked. 

The question arises whether extirpation of the tumor 
should be attempted in cases of a tubercular nature and 
in those in which gumma is suspected. In tu*bercle of the 
cord, even the successful removal would in all probability 
prolong life but very little, so that surgical interference is 
scarcely warranted ; and yet in a case that is otherwise fa- 
vorable for operation there seems to me to be sufficient rea- 
son to interfere, if there are no other symptoms of general 
tuberculosis. I am in favor of removing gummata if the 
tumor has resisted, as it generally does, every form of treat- 
ment, provided such treatment has been carried out relig- 
iously for a period of from three to four weeks without any 
sign of improvement. I consider Horsley's advice entirely 
sound, that if a tumor has resisted every form of treatment 
for a sufficient length of time, and if the conditions are 

* Since the above was written, Starr has reported several cases in which the neo- 
plasm was removed successfully ; but not a single case can be said to have been cured. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 373 

favorable for surgical interference, the proper surgical pro- 
cedures should be resorted to, and that, too, without much 
delay, for delay is far more serious under these circum 
stances than prompt and proper surgical interference would 
be. 

SYRINCxOMYELIA AND GLIOSIS OF THE CORD. 

Syringomyelia, or, more properly speaking, " myelosyringosis," is a form 
of disease that is rare enough in the adult, and still rarer in children. Its 
symptoms resemble somewhat those of tumor of the cord, of amyotrophic lat- 
eral sclerosis and of progressive muscular atrophy. As the name indicates, 
the disease is due to cavity formation in the spinal cord. This may be the 
result of a congenital abnormality of structure, or it may be due to a glioma- 
tous process starting in the vicinity of the central canal, and causing a de- 
struction of tissue. (See Hoffman's articles.) 

We need not discuss whether the one or the other mode of origin is the 
more frequent, and on this point opinions still vary. The cavity is, as a rule, 
largest in the cervical segments, but may be continued up into the medulla and 
downward into the dorsal and lumbar segments. 

Symptoms. — Less than fifteen years ago syringomyelia was considered a 
mere curiosity without any special practical importance ; but the investigations 
of Schultze and of Kahler have enabled us to recognize the disease by a very 
definite set of symptoms. The symptoms will naturally vary according to the 
extent of the cavity ; but as the cavity is generally most developed, and has 
its beginning in the cervical, region, the symptoms are first observed in the 
upper extremity and generally around the shoulder. 

We may divide the symptoms into three groups : The first group consists 
of trophic disturbances, affecting the skin, its subcutaneous tissues, and the 
bones. Glossiness of skin, particularly of the fingers, deep fissures, felons — 
which are sometimes painless — and phalangeal necrosis, with marked distor- 
tion of the fingers, are the most frequent. Patients also complain of burning, 
prickling feelings, and of a sensation of numbness. These various disturb- 
ances are associated at an early date with the second group of symptoms, con- 
sisting of partial disturbances of sensation. There is either a diminution or a 
complete abolition of the sense of pain and of temperature, while the sense of 
touch and of muscular innervation remains absolutely normal. 

The third group of symptoms includes a progressive atrophy of the mus- 
cles, beginning in the small muscles of the hand, and gradually involving the 
forearm, the arm, and the shoulder muscles. The resemblancce to the Aran- 
Duchenne type of progressive muscular atrophy is very great (see Chapter 
XXIII.), and it is only by the association of this progressive muscular atrophy 
with dissociated disturbances of sensation, and with the trophic symptoms 
described above, that we can differentiate between syringomyelia and progres- 
sive muscular atrophy, as well as amyotrophic lateral sclerosis. In progressive 
muscular atrophy of the Aran-Duchenne type, there are no marked disturb- 
ances in the skin and subcutaneous tissue ; and in amyotrophic lateral sole- 



374 



THE NERVOUS DISEASES OF CHILDREN 



rosis there are no disturbances of sensation, and no marked trophic symptoms,, 
but in this disease and in syringomyelia the reflexes may be exaggerated. 

In syringomyelia other symptoms occur, which will depend largely upon 
the extent of the cavity formation. If the cavity encroaches very largely 
upon the anterior gray matter, there will be a large amount of flaccid atrophy 
and paralysis, the electric reactions will be disturbed, and the reflexes will be 




Fig. 89. — Section through Seventh Cervical Segment, showing Gliosis of Cord. 

(Herter.)* 



diminished in the parts governed by the affected region. Fibrillary tremor is 
present in some cases. All these symptoms are generally most pronounced 
in the upper extremities, since the cavity is greatest in the cervical region, and 
if the lateral columns in the cervical region are involved, we may expect a spastic 
paralysis of the lower extremities with increased reflexes, rigidity, and con- 
tractures. Cutaneous reflexes are, at times, normal, at other times diminished 
or entirely absent. Hyperidrosis or hypidrosis (unilateral or bilateral) have 
been reported. In the cervical cases a narrowing of the palpebral fissure and 

* This and the following figure are reproductions of specimens prepared and pho- 
tographed by Dr. Herter, and kindly furnished me for use in this book. 



TUMORS OF THE SPINAL CORD AND ITS MENINGES. 375 

contraction or dilatation of the pupils must be added to the possible symptoms 
of this curious disease. If the affection extends into the medulla and pons, 
disturbed sensation in the area supplied by the trigeminus is present, together 
with atrophy and paralysis of the tongue, hoarseness, difficulty of deglutition, 
and disturbances of the senses of taste and hearing. Other cranial nerve 
nuclei may be involved ; nystagmus may be present ; polyuria and increased 
salivation have also been noticed. Pains and rigidity in the upper portion of 







FlG. 90.— Section through Part of Fourth Dorsal Segment. The central canal is in- 
vaded by glia cells and surrounded by groups of cells and nuclei resembling those of 
the ependyma. (Herter.) 



the spinal column are not infrequent, and scoliosis or a combination of scoli- 
osis and kyphosis may also be present. Vesical and rectal disturbances are 
at times superadded. 

Syringomyelia is practically a disease of adolescence and of adult life, but 
some of its symptoms can at times be traced back to the second decade of 
life. The earliest case which I have seen was in a woman of twenty, in 
whom the first symptoms began at the age of fifteen. It is interesting to 
note, however, in this connection, that a disease which is intimately related 



376 THE NERVOUS DISEASES OF CHILDREN. 

(at least in its pathology) to syringomyelia, has been observed by Dr. Herter 
in a child one year of age. Through his kindness I am enabled to insert a 
summary of his (unpublished) case, which he reported to the New York Neu- 
rological Society. 

Three months before the child was examined the right arm and hand 
began to be weak, and the head drooped forward. The examination brought 
out the following points : Complete loss of powe^and atrophy of muscles in 
both arms and shoulders ; the muscles were flabby ; there was slight move- 
ment of hands and fingers ; rigidity of neck ; the knee-jerks were increased, 
especially on the right side ; ankle clonus was elicited on both sides ; there 
was an irregular rise of temperature, ranging between ioo° and 105 F. daily. 
The child died two weeks after examination. 

The post-mortem examination revealed considerable enlargement of the 
right half of the medulla oblongata and of the cervical cord extending to the 
eighth cervical segment. There was some enlargement from this point to the 
sixth dorsal ; below this the cord appeared to be normal. In the upper cer- 
vical segments the nervous structures of the spinal cord were replaced by a 
new growth ; in the lower cervical region the pressure effects of the new 
growth were evident. At about the level of the seventh cervical segment 
the central canal w T as lined with an unbroken layer of columnar ependymal 
cells, except at the posterior wall, which was broken through by a mass of 
larger glia cells, which partially filled the canal. (Fig. 88.) The glioma in- 
vaded nearly the entire cross-section of the cord, but undoubtedly originated 
in the vicinity of the central canal. 

The case is not unlike one reported by K. Miura ; Sokoloff described a 
glioma of the medulla, with cavity formation, in a boy, aged five years. 

BIBLIOGRAPHY. 

Glaser : Arch, fur Psychiatrie, vol. xvi., p. 89. 

Gowers and Horsley : Med. Chirurg. Trans., vol. lxxi., 1888. 

Hayem : Arch, de Physiologie Norm., etc., 1873. 

Herter : Journal of Nervous and Mental Disease, 1890, p. 631. 

Hoffman (Syringomyelia) : Volkmann's series, No. 20, 1891 ; also Zeitschr. f. 

Nervenheilk. , vol. iii., 1892. (Contains literature.) 
Kolitz : Wiener Med. Blatter, 1885, pp. 41-44. (On Tumors of Spinal Cord 

in Children.) 
Laqueur: Neurologisches Centralblatt, 1891. 
Lloyd : In American Text-Book of Diseases of Children. 
Mills and Lloyd : In Pepper's System of Medicine, vol. v., p. 1091. 
M. Miura : Virchow's Arch., vol. cxvii., p. 435. 
K. Miura : Ziegler's Beitrage, vol. xi. 

Sachs: Journal of Nervous and Mental Disease, vol. xiii., No. 11, 1886. 
Schultze, Richard : Arch. f. Psychiatrie, 1885, vol. xvi., p. 592. 
Sokoloff: Deutsch. Arch. f. kl. Med., vol. xli., 1887. 
Thorburn : Surgery of Spinal Cord, Case xlix., p. 168. 
Walter Vought : New York Medical Journal, June n, 1892. 
Williams : Deutsches Archiv. f. kl. Medicin, vol. xxv., 1880. 






CHAPTER XXII. 

HEREDITARY OR FAMILY DISEASES OF THE SPINAL CORD. 

Hereditary or family affections of the spinal cord con- 
stitute an important group of diseases. They are the re- 
sult of defective development, or possibly of arrest of de- 
velopment, of various parts and systems of the spinal cord. 
Of these disorders hereditary ataxy, or Friedreich's disease, 
is the best known. A few years ago Nonne and Marie de- 
scribed a type of disease which bears a close resemblance 
to hereditary ataxy, and for which Marie proposed the term 
Hereditary cerebellar ataxy (Heredo-ataxie cerebelleuse). 
In Friedreich's disease the morbid process is restricted to 
the posterior columns or to the posterior and lateral col- 
umns ; in Marie's disease there is in all probability a defec- 
tive development of the cerebellum and of the tracts con- 
nected with it. Congenital or hereditary defect of the 
pyramidal tracts also leads to another distinct group of dis- 
eases which we may term Hereditary spastic paralysis ; and 
Hoffman has still more recently described an hereditary 
form of progressive muscular atrophy of spinal origin in 
which the chief defect was found in the anterior gray mat- 
ter of the cord. These hereditary diseases suggest a clinical 
relationship to syphilitic disease of the spinal cord (which 
may be inherited, though not hereditary) and to some forms 
of cerebral birth palsies. Moreover, some of these hered- 
itary affections are distinctly cerebro-spinal, both as regards 
the distribution of the lesions and the character of the symp- 
toms. It is no easy task to separate these various forms 
from one another and to bring order out of chaos. The 
family form of progressive muscular atrophy will be con- 
sidered to much greater advantage in connection with the 
other forms of progressive muscular disease. 



378 



THE NERVOUS DISEASES OF CHILDREN 



HEREDITARY ATAXY. — FRIEDREICH'S DISEASE. 

Hereditary ataxy, or Friedreich's disease, as it is called 
in honor of the physician who gave the first satisfactory ac- 
count of it, is a family af- 
fection. Several genera- 
tions are often affected by 
it ; but naturally we may 
find every now and then 
that the disease occurs 
in one or several mem- 
bers of a family without 
any history of a similar 
trouble in the patient's 
ancestry. It is not nec- 
essary, therefore, to speak 
of such cases as non- 
hereditary forms of Fried- 
reich's ataxia, as an Eng- 
lish writer has recently 
done, for the disease must 
invariably have a starting- 
point, and the patients 
under observation may 
indeed be the first of a 
long series that are to 
follow. The children who 
have come under my spe- 
cial notice in three dif- 
ferent families gave no 
history of any similar 
trouble in preceding gen- 
erations. 

The marked tenden- 
cy to the occurrence of 
this disease in families is 
shown, as Gowers states, 
by the occurrence of sixty-five cases in nineteen families, 
giving an average of rather more than three to each fam- 




Fig. 91. 



-Case of Hereditary Ataxy (Fried- 
reich's Disease). 



HEREDITARY DISEASES OF THE SPINAL CORD. 



379 



ily. The number has varied from two to as many as eight 
in a single family. Both sexes are about equally liable, but 
sometimes a special predilection is shown in favor of one 
or the other sex. In one family of nineteen, two males were 
affected with the disease and seventeen females escaped. 
All the cases which I have observed were in boys, the sis- 
ters of these escaping entire- 
ly unharmed. The disease 
comes on, as a rule, very early 
in life, most of the symptoms 
being fully developed before 
the age of fourteen. In the 
family referred to by Everett 
Smith, of Boston, the symp- 
toms of ataxic paraplegia 
were observed in the father 
at the age of sixty-six, but it is 
questionable whether his was 
a typical hereditary ataxy, 
and it is safer to adhere to 
the belief that Friedreich's 
disease will always appear 
early in life. If several mem- 
bers of one family are affected 
the disease is developed in 
all at about the same age. 

Symptoms. — The symp- 
tomatology of hereditary at- 
axy is now as firmly estab- 
lished as that of almost any 
spinal disease. Friedreich 
described most of the symp- 
toms in 1861. The French 
school, including Charcot, 
Vulpian, Brissaud, and Ma- 
rie, have given careful study to this disease. In England. 
Carpenter, Gowers, Ormerod, and Bury described cases of 
true hereditary ataxy ; while in this country the chief con- 
tributions to this subject have been made by Smith. Ham- 
mond, Seguin, Dana, Church, Sanger Brown, and [nglis. 




Fig. 92. — Same Patient as in Fig. 89. 
Marked atrophy of the muscles about 
the shoulder girdles (infra-spinati, su- 
pra-spinati, rhomboids, and others). 



380 THE NERVOUS DISEASES OF CHILDREN. 

At a very early age children suffering from Friedreich's 
disease exhibit peculiarities in walking and standing. The 
child walks with its legs widely apart, in an uncertain, hes- 
itating fashion, reminding one very strongly of a combined 
ataxic and cerebellar gait. There are in addition in some pa- 
tients oscillatory movements of the head, which remind one 
a little of multiple sclerosis, and a little of such movements 
as we sometimes see in cases of senile degeneration. When 
the patient is asked to stand still, with his feet closely ap- 
proximated, he soon begins to stagger, as tabic patients do, 
and as soon as his eyes are closed falls to the ground un- 
less properly supported. Marie states that Romberg's 
symptom is ordinarily absent, but it has been most distinct- 
ly present in those subjects whom I have had occasion to 
examine. In addition to these disturbances in gait and in 
standing we also find a coarse tremor present in many of the 
cases, and in some a condition which is not so distinctly 
tremulous as it is ataxic and awkward. If the patient is 
asked to take hold of a pencil, to raise a glass of water to 
his lips, or to attempt to write, he seizes upon the object 
that he is to hold in a distinctly ataxic way and uses the 
hands awkwardly, sometimes after the fashion of extremely 
choreic movements. It is this combination of tremor, of 
awkward movements, and of choreic manifestations that 
gives to these children their characteristic aspect. There 
has been some discussion as to the occurrence of actual pa- 
ralysis ; in those whom I have examined there was in the 
first few years only a very slight loss of muscular power 
both in the upper and in the lower extremities. In ad- 
vanced stages the grasp is almost nil, and the lower extrem- 
ities may be so thoroughly paralyzed that it is impossible 
for the patient to lift a leg or even to move a toe, but in 
this respect the disease evidently varies very much. 

Sensation may be interfered with to a slight degree, but 
not nearly so markedly as in the tabes dorsalis of the adult. 
Lightning pains are infrequent, although Charcot insists that 
in some instances these occur quite as typically as in tabes. 
Anaesthesia and analgesia are rarely present. Several 
French authors have described cases in which hemianes- 
thesia occurred ; but Marie is inclined to attribute this to the 



HEREDITARY DISEASES OF THE SPINA I CORD. 38 1 

association of hysteria with Friedreich's disease. The mus- 
cular sense is rarely affected, and in the persons whom I 
have examined for the purpose of determining the presence 
or absence of this sense, I have found it invariably present, 
even in those in whom there was marked inco-ordination of 
the upper extremities. 

Reflexes. — The superficial reflexes are, as a rule, pre- 
served ; but the deep reflexes are generally absent. It is 
this absence of the deep reflexes, particularly of the knee- 
jerks, that brings out in some subjects the very closest re- 
semblance between Friedreich's disease and tabes dorsalis. 
In cases in which the reflexes are exaggerated there is good 
reason to doubt whether the disease corresponds to that 
described by Friedreich ; * but there is no reason why in 
those forms in which the affection involves both the posterior 
and lateral columns the knee-jerk should not occasionally 
be augmented, and it is probably in view of such mixed types 
that Gowers has spoken of a form of hereditary ataxic para- 
plegia under the heading of Friedreich's disease. It is one 
thing to maintain a type as originally described by its dis- 
coverer, and quite another thing to deny that any departure 
from such a typical series of symptoms implies an entirely 
different order of disease. 

Difficulties of micturition and of defecation occasionally 
occur, but are not so constant an accompaniment of Fried- 
reich's disease as they are of tabes ; in the earlier stages of 
the disease, at all events, the vesical and rectal reflexes re- 
main intact. 

Trophic and vasomotor disturbances are also rare, if we 
except a general discoloration of the skin and coldness of 
feet and hands. Marie and other French authors refer to 
the occurrence of a special form of club-foot which they 
have observed in these cases of hereditary ataxy. Marie 
states that the foot is shortened ; that the anterior portion 
is particularly broad if viewed laterally ; the foot is in the 
condition of a pes cavus. The toes are hyperextended and 
have a claw shape. This deformity of the toes is said to 
have been observed as one of the early symptoms bv parents 
in whose families this special disease has been hereditary. 

* Some of these may belong to the Heredo-ataxie cerebelleuse of Marie. 



382 



THE NERVOUS DISEASES OF CHILDREN. 



Muscular atrophy constitutes an important symptom of 
this disease. It is most distinctly visible in the shoulder 
and pelvic girdles. It was so prominent a feature in one of 
my patients that on first examination I was inclined to re- 
gard the trouble as one of an hereditary form of progressive 
muscular atrophy, until the examination of the brother, who 
was in a more advanced stage of the disease, proved to me 
beyond the possibility of a doubt that both cases were typ- 
ical of Friedreich's disease. In the boy represented in Fig. 
90, the excessive atrophy must be attributed to an addi- 
tional involvement of the gray matter of the cord. 




Fig. 93. — Deformity of the Feet in a Case of Friedreich's Disease ; Hyperextension 
of the Toes and Club Foot. (Marie.) 



We have now to consider a further set of symptoms 
which seem to me to prove the cerebro-spinal character of 
the disease. N}'stagmus occurs in many of these cases, but, 
as a rule, does not appear until several years after the onset 
of the first symptoms. The nystagmus can often be elicited 
only upon extreme use of the ocular muscles. In this con- 
nection it may be well to remember that nystagmus upon 
extreme movements occurs in not a few healthy individuals, 
and that the presence of such movements in a child of a 
family affected with Friedreich's disease need not neces- 
sarily indicate the development of this serious trouble. 
Ocular palsies occur, but are rare. The Argyll-Robertson 



HEREDITARY DISEASES OF THE SPINAL CORD. 383 

pupil is as regularly absent in Friedreich's disease as it is 
present in cases of tabes. Optic atrophy and amblyopia 
have not been described in these cases. All the other 
senses remain entirely unaffected. 

Among cerebral disorders occurring in the course of 
Friedreich's disease, vertigo and dizziness are extremely 
frequent. These may in part be due to the oscillatory 
movements of the head. In the earlier years of the disease 
the intelligence is not affected, except that the children can- 
not be educated at schools as other children are, and there- 
fore remain backward. As the disease progresses a distinct 
defect of intelligence is noticeable, and a condition of semi- 
idiocy may be the result. The patients appear, however, 
far more idiotic than they really are, and this appearance is 
caused by the peculiar stupid expression of face, and above 
all by the disturbances in speech, to which we shall now 
refer. The speech reminds one a little of multiple sclerosis 
in that it is slightly scanning, but it is at times hesitating 
and jerky, at other times slow, deliberate, and awkward. 
On the whole, I find Marie's simile a good one, who com- 
pares it with the cerebellar gait, since it is, as he says, awk- 
ward, uncertain, and vacillating. All these symptoms are 
not developed until after the lapse of years. At first the 
peculiar gait and position are noticeable, later on the inco- 
ordination of the upper extremities and the difficulties of 
speech become more marked ; then, after the lapse of a 
longer or shorter period of time, the patients become help- 
less and may remain bedridden or confined to their chairs 
for a period of ten and even twenty years. The gradual 
development of cerebral symptoms denotes the continuous 
progress of the disease. 

In the following histories of two brothers the chief symptoms of Fried- 
reich's disease are well illustrated : 

Case I. — H. S , twenty-three years of age, second child.* 

Family History : Parents healthy ; not related to one another ; no neu- 
rotic taint in the ancestry. Thirteen children, all well and normal in every 
respect except this patient and his younger brother (Case il.). An older 
brother and two younger sisters of the patient and his parents were examined 

* I am particularly indebted to Dr. L. Stieglitz, who visited this patient at his home 
in the country ; the patient's brother (Case II.) was examined at my clinic. 



384 THE NERVOUS DISEASES OF CHILDREN. 

with regard to speech, gait, and ocular muscles ; no anomalies discovered 
in them ; speech fluent. 

Patient was well till his eighth year, when he was knocked down by a 
cow ; but was not injured. First symptoms came on a year later ; the 
waddling gait of the boy attracted attention ; his gait grew steadily worse, he 
often " stumbled over himself " and fell to the ground ; if he attempted to run 
he would reel as though intoxicated. His mother believes his speech began 
to change when he was thirteen years old ; it then became slow, monot- 
onous, somewhat jerky, less distinct than it used to be. About three years 
ago he suffered for many months from severe shooting pains in both legs ; 
the pains gradually left him ; the last two years he has been free from pain. 
During this period other symptoms were developed ; bladder trouble set in and 
bowels were constipated ; patient could not retain his water and wet his bed, 
especially at night, for several weeks. For the past few years the patient has 
been perfectly helpless. 

Examination (July 3, 1893) : Patient sits in a chair and is utterly helpless. 
Expression of face idiotic ; intelligence, however, but very little below the 
average. He is not able to move his legs, feet, or toes. He moves his 
arms when asked to do so in a distinctly ataxic, awkward fashion. Muscles 
of arms very well developed ; grasp strong ; no weakness discoverable in any 
of the groups of muscles in the upper extremities. The first interosseous 
space on either side depressed. Electrical reaction (faradic current) of all the 
nerves and muscles of the arms unimpaired. No disturbance of sensation in 
either arm. The legs seem to consist of nothing but skin and bones. Cir- 
cumference of calf (both legs) 23 ctm. (about ten inches). Both feet 
large, deformed (pes equino-varus), blue and slightly swollen, reminding one 
of the blue swelling of lead palsy. Slight movement of the thighs at the hip- 
joints, otherwise complete paraplegia ; with a strong faradic current response 
is quick ; contractions of all of the muscles of the thigh and legs were elicited 
on direct and indirect excitation. Knee-jerks are absent, plantar reflex 
present. Sensation for touch and of temperature slightly impaired, especially 
in the feet. Pupils react promptly to light. Vision apparently unimpaired. 
Very marked ataxic nystagmus. Speech very slow, monotonous, at times 
jerky. 

Case II. — L. S (Figs. 89, 90), twelve years of age ; ninth child. Per- 
fectly well till seven years old, when mother first remarked that he waddled 
in his walk ; he could walk quite a distance at that time without feeling 
fatigued ; had great difficulty in going up and down stairs ; became slowly 
but steadily worse. About two years ago his speech began to change ; he 
began to speak more slowly, and in a monotonous tone. Went to school for 
two years and did well. No pains ; no bladder trouble. His mother says 
he never lets things drop out of his hands, when he once has hold of them, 
but in trying to grasp a glass or cup he would often knock against it and 
throw it over before getting a firm grip upon it. The last few months the 
boy has been suffering from malaria. 

Examination (spring of 1893): Ill-nourished, emaciated boy; shy, and 
when addressed appears to be stupid, but his intelligence is quite up to the 






HEREDITARY DISEASES OF THE SPINAL CORD. 



385 



average. The first interosseous space of both hands, the fossae infra- and 
supra-spinatae much depressed ; winged scapulae. Entire muscular system 
poorly developed. 

Motor Sphere : The boy's gait is highly ataxic (cerebellar-ataxic), his legs 
are spread wide apart, both in standing and in walking ; staggers as though 
intoxicated, stumbles over his own feet, but manages to cover ground fairly 
well. Romberg's symptom. Knee-jerks absent. 

Muscles of limbs, of back, and thorax thin and atrophic ; grip fairly good ; 
the power of his muscles much greater than their volume would lead one to 
expect. No true paresis in any single group of muscles. There is distinct 
weakness with atrophy in the muscles of the shoulder, the deltoid, pectoralis, 
infra- and supra-spinati, and the rhomboids ; the lower half of trapezius is 
atrophic, the upper portion fairly well preserved. With a strong current 
(faradic) all but the most atrophic muscles were made to contract on direct 
and indirect excitation ; the rhomboids and the muscles of the shoulder-blade 
responded feebly to the strongest currents. Galvanic formula not reversed, 
but excitability much diminished ; ataxy of arms and legs, and no disturbance 
of sensation. Slight nystagmus upon extreme lateral movements of eyeballs. 
Speech slow, monotonous — almost scanning. 

One year after this examination the boy could scarcely walk ; is now in a 
hospital suffering from mitral insufficiency ; is extremely emaciated ; intelli- 
gence not markedly impaired. 



Differential Diagnosis. — The symptoms of heredi- 
tary ataxy are so distinct that one would suppose confusion 
with other diseases a sheer impossibility, yet mistakes do 
frequently occur. The disease bears closest clinical re- 
semblance to tabes. The age of the patient and the occur- 
rence of the disease in families will serve to distinguish it 
from tabes dorsalis, but if a person in advanced age is ex- 
amined for the first time, special attention must be directed 
to the following points : 



Tabes. 

Argyll-Robertson pupil present. 

No nystagmus. 

No tremor of the head. 

No peculiarities of speech. 

Inco-ordination of upper extremities 

rare. 
Lightning pains and various crises. 
Ataxic gait. 
No defect of intelligence. 

25 



Friedreich's Disease. 

Argyll-Robertson pupil absent. 
Nystagmus present in later years. 
Tremor of the head present. 
Speech altered. 
Inco-ordination the rule. 

No such pains and crises, as a rule. 
Ataxic-cerebellar gait. 
Intelligence defective in later years. 



386 THE NERVOUS DISEASES OE CHILDREN. 

Iii the above parallel columns the symptoms which both 
have in common have been omitted, such as absence of re- 
flexes and Romberg's symptom. 

Multiple sclerosis can be distinguished easily from 
Friedreich's disease by the excessive exaggeration of the 
reflexes, by the intention tremor, by the marked spasticity 
of the gait, by the occurrence of ocular palsies, all of which 
rarely occur in cases of Friedreich's disease. Confusion 
might arise from consideration of the similarity in speech 
and the occurrence of nystagmus. Marie includes in the 
consideration of the differential diagnosis ordinary chorea, 
but it seems to me that no one but a very obtuse person 
could be misled by the occasional occurrence of awkward 
and choreic movements in Friedreich's disease, for in no 
other respect are the two diseases at all similar. 

Confusion may more readily occur with cerebellar diseases, and Schultze 
has recently, in a satirical vein, in answer to Senator, called attention to the 
fact that, after all, cases that would seem to be of the cerebellar order are 
altogether different from the type which Friedreich described. I can ap- 
prove of this remark of Schultze, for I have myself seen a case of defective 
cerebellar development * in which there was a superficial resemblance be- 
tween them and cases of Friedreich's disease, and yet a careful considera- 
tion of the conditions was sufficient to distinguish between the two sets 
of diseases. 

Pathological Anatomy. — The morbid anatomy of 
Friedreich's disease has not, as yet, been satisfactorily de- 
termined. Few autopsies have been made, and in some of 
the cases which have been examined post mortem, the symp- 
toms have varied so much from the type, as laid down by 
Friedreich, that the results of the examination have helped 
to obscure rather than to clear up the doubts regarding the 
true anatomical changes of the disease. A number of au- 
thors have called attention to the attenuation of the entire 
cord in cases of hereditary ataxia, the diameter of the cord 
being but three-fourths or two-thirds of the normal. This 
attenuation is most marked in the dorsal region ; but 
whether it is due to a disappearance of nerve-fibres, to the 
retraction of sclerotic tissue, or whether it is simply a de- 
fect of development, has not been clearly made out. Any 

* See Chapter on Conditions due to Defective Development of the Brain. 



HEREDITARY DISEASES OF THE SPINA I CORD. 



387 



one of these three causes may be operative in some cases, 
or a co-operation of all three is a possibility in others. The 
one fact that is absolutely indisputable is that on micro- 
scopical examination a sclerosis of the spinal cord is found, 
involving at different levels, or at one and the same level, 
various systems of the cord. This sclerosis affects most 
frequently the posterior columns or the lateral columns, or 
both together, and hence the symptoms vary between those 
of a pure posterior spinal sclerosis and those due to a 




Fig. 94. — Section through Middle Dorsal Region of a Case of Friedreich's Disease. 
(After Blocq et Marinesco, from Marie's treatise.) A, small degenerated area, prob- 
ably Gowers' tract ; B, degeneration in lateral columns ; C, direct cerebellar tract ; 
D, column of Burdach ; E, column of Goll — both degenerated ; G, a strand of 
healthy fibres. 



postero-lateral sclerosis, resembling the symptoms of the 
ataxic paraplegia of the adult. 

Taking up the lesions of Friedreich's disease in detail, 
we may note the following distribution : 

1. In the posterior columns, in a number of the cases, the columns of 
Goll are sclerosed throughout their entire length from the lower part of the 
spinal cord to the calamus scriptorius. The column of Burdach is also in- 
volved throughout the greater part of its course; but, according to Marie, the 
intensity of this sclerosis varies in different levels of the cord. The external 
portion of the column of Burdach, near the posterior horns, is generally ex- 
empt from sclerosis ; in the cervical region the sclerosis of the column of 
Burdach begins to diminish, and disappears altogether in the vicinity of the 
medulla oblongata. 



388 



THE NERVOUS DISEASES OF CHILDREN. 



2. The direct cerebellar tract is involved from its beginning in the lower 
dorsal region to the upper cervical region. Toward this latter part it dimin- 
ishes considerably, but is most marked in the upper dorsal segments. Sev- 
eral observers have noted that the lesion extends beyond the direct cerebellar 
tract, and involves the antero -lateral tract, or tract of Gowers ; and Marie 
goes to the extent of stating that he thinks this involvement of the antero- 
lateral tract an almost constant feature of Friedreich's disease. 

3. As for the lateral columns, the opinions of authors differ somewhat. 
Marie is not willing to allow the regular involvement of the lateral columns, 
while he concedes that the diseased fibres occupy the position of the crossed 
pyramidal tract. He does not believe that they represent the fibres of that 




Fig. 95. — Section through a Cervical Segment from a Case of Friedreich's Disease. 
(Schultze.) Degenerated areas in posterior and lateral columns and in anterior col- 
umn (left half of figure). Posterior root fibres also degenerated. 



tract. His reasons for maintaining this are, first, that the lesion of the lateral 
columns in Friedreich's disease diminishes considerably from below upward to 
the level of the lower portion of the medulla oblongata, the very reverse of 
which would occur if these fibres were part of the pyramidal tract. Secondly, 
that on a transverse section of the spinal cord the localization of the lesion 
of the lateral column does not correspond exactly to the site of the lesion of 
the pyramidal tract proper. Third, nothing in the clinical appearances of Fried- 
reich's disease reminds one of the symptoms which are a constant accom- 
paniment of changes in the pyramidal tract. The only explanation which 
Marie is able to give for the fibres that are affected in the lateral region is 
that they are probably fibres which connect the direct cerebellar tract with the 
antero-lateral tract of Gowers. Of the reasons advanced by Marie, the first 
seems to me to be the only one that will bear close scrutiny ; the second 
is scarcely susceptible of proof, and as for the third, it is sufficient to state 



HEREDITARY DISEASES OF THE SPINAL CORD. 389 

that the disease attacks both the posterior columns and the pyramidal fibres 
at various levels. 

4. A difference of opinion also exists as regards the marginal zone of 
Lissauer, as many authors claim to have found these zones entirely intact, 
while others claim that an affection of these fibres constitutes a regular feat- 
ure of Friedreich's disease. At all events the affection of Lissauer's tract is 
not nearly so constant or so early a feature of the disease as it is in the spinal 
ataxia in the adult. 

The morbid anatomy of Friedreich's disease is not exhausted with this 
statement regarding the behavior of the white fibres. The gray matter is 
affected as well. The columns of Clarke exhibit a loss of fibres very much 
like that in tabes, as well as a considerable diminution of their cells, which 
are also smaller and devoid of their long-cell processes. In the posterior 
horns there, is a diminution of volume, and also a decided diminution in the 
number of cells. The anterior horns have been found altered by some, 
Friedreich being among them, and the cells may be atrophied. There is 
no reason to doubt this occurrence in view of a considerable atrophy of the 
muscles which occurs in some of these cases, as in one of my own. 

Some changes have been noticed in the ependyma of the central canal, but 
these are not sufficiently constant to be considered a regular morbid feature 
of Friedreich's disease. Some have also insisted that the spinal meninges are 
involved, while others dispute such involvement. If they are affected at all 
it is in the vicinity of the posterior columns. Little more can be said of the be- 
havior of the posterior roots, although Blocq and Marinesco insist that they are 
as frequently altered in Friedreich's disease as they are in ordinary tabes. The 
peripheral nerves are supposed to be entirely normal in Friedreich's disease, 
and to this fact Dejerine and others attributed the entire absence of fulgurating 
pains in hereditary ataxy, while they are universally present in tabes dorsalis. 

The question remains as to the nature of the morbid proc- 
ess which gives rise to Friedreich's disease? A number of 
views have been propounded. Some have claimed it to be a 
mere arrest of normal development, while others regard the 
morbid process as a true sclerosis. Dejerine and Letulle, on 
the strength of certain peculiar fibres which they have found, 
and which they consider similar to fibres discovered bv 
them in the cortex of epileptic patients, are inclined to re- 
gard the sclerosis of hereditary ataxia as a sclerosis of the 
neuroglia. They insist that neither the connective-tissue 
septa nor the blood-vessels are at all altered, and that the scle- 
rosis is therefore very different from the vascular sclerosis 
which so often involves the pyramidal tracts and the direct 
cerebellar tract ; but later investigators, among them Wei- 
gert, have disproved these views of Dejerine and Letulle. 



390 THE NERVOUS DISEASES OF CHILDREN. 

They regard the sclerosis of Friedreich's disease as in no- 
wise different from the ordinary sclerosis excepting in this, 
that it has been developed at an unusually early period. 
Weigert, in his recent studies on the neuroglia, concludes 
that in the ordinary spinal sclerosis, as it occurs in tabes, in 
multiple cerebro-spinal sclerosis, and in amyotrophic lateral 
sclerosis, the proliferation of the neuroglia is a much more 
marked feature than in hereditary ataxy. The refutation 
of this theory of Dejerine and Letulle does not, how- 
ever, help us in the recognition of any true theory of the 
disease, and for the present we must confess that we do not 
know why or how the sclerosis of Friedreich's disease is 
developed. 

Prognosis. — The prognosis of Friedreich's disease is in- 
variably grave. In the course of four to six years the patient 
becomes crippled, unable to move about except in a chair, 
awkward in speech, and more or less demented. Unfortu- 
nately the disease does not lead to a fatal issue within a 
reasonable period of time, some of the patients attaining the 
age of forty years and more. 

As for treatment, no special method need be recom- 
mended. The progress of the disease cannot be stayed, the 
relief of pain constituting the only reason for active inter- 
ference on the part of the physician. The ordinary anti- 
neuralgic remedies may be employed to this end. In addi- 
tion, suspension, electrical treatment, massage, and the like, 
may be ordered, in the discretion of the physician. 

HEREDITARY ATAXY (CEREBELLAR TYPE ; TYPE NONNE-MARIE). 

Under the title " Heredo-ataxie Cerebelleuse " Marie collected a number 
of cases which bear a very close resemblance to Friedreich's disease, yet pre- 
sent several characteristic and different symptoms. Titubation, Romberg's 
symptom, tremor of the head and of the extremities, choreiform, awkward 
movements, particularly of the arms and hands, and nystagmus, are symp- 
toms common to Friedreich's disease, and to the form we are now describing. 
The cerebellar form of hereditary ataxy comes on, as a rule, at the age of pu- 
berty or later. The knee-jerks, instead of being diminished, are increased. 
There are marked ocular symptoms, the pupils fail to react to light and dur- 
ing accommodation. There is diplopia in some cases, color-blindness in 
others. Contraction of the visual field has been observed, and amblyopia, due 
to atrophy of the optic nerve, is a typical symptom. There are also marked 



HEREDITARY DISEASES OE THE SPINAL CORD. 39 1 

disturbances of sensation. The deformities of the foot and kyphosis, noted in 
Friedreich's disease, are never present in the cerebellar type. 

This symptomatology, as developed by Marie, reveals a very striking re- 
semblance between these cases of hereditary cerebellar ataxy and a form of 
family disease described by Nonne. In all probability Nonne's and Marie's 
descriptions refer to the same type. While the latter deserves credit for 
naming the disease, it seems to me just that Nonne's name should be con- 
nected with this type. There is also a very close resemblance between this 
hereditary cerebellar ataxy and some of the cases which have been described 
under the heading of " Hereditary Spastic Paralysis." Senator's views regard- 
ing Friedreich's disease may in part be explained by the recognition of this spe- 
cial order of disease, and there would surely be a possibility of confounding 
this type of hereditary ataxy with the cerebellar type of multiple sclerosis 
which Charcot has described. 

It is too early to report definitely upon the pathology of hereditary cere- 
bellar ataxy. If we are correct in considering the cases described by Marie 
and Nonne as representatives of the same type, .the post-mortem findings in 
one of Nonne's cases will give some clew to the pathology of this variety of 
disease. In Nonne's case the cerebellum was small, and there was a simple 
diminution of tissue without sclerosis or degeneration. The spinal cord was 
much attenuated throughout its entire length. Under the heading of " He- 
reditary Ataxy and Atrophy of the Cerebellum " Menzel described a condition 
in which there was a combined systemic disease of the spinal cord, and in 
addition atrophy of the cerebellum, pons, and medulla. Two French authors, 
Royet and Collet, have reported a case which was diagnosticated during life 
as multiple sclerosis, in which there was found on post-mortem examination 
a lesion of the cerebellum and of the cerebellar tracts in the pons. It is 
possible that some of the cases which have been regarded as a form of he- 
reditary multiple sclerosis may come under this same heading. I recognize 
that there is great danger in multiplying types of disease. The form de- 
scribed by Marie and Nonne should in justice to these two authors be given 
due consideration ; but I believe it better for the present to regard it as a 
variety of hereditary ataxy.* 



HEREDITARY SPASTIC PARALYSIS (SPINAL, CEREBRAL OR 
CEREBRO-SPINAL TYPES). 

During the past few years a number of family affections 
have been described by Homen, Newmark, Pelizaeus, Strum- 
pell, Freud, Erb, and myself, in all of which a spastic 
rigidity, or a spastic paralysis affecting the lower extremi- 
ties chiefly, has been the most prominent symptom. In 
some cases the spastic symptoms were evidently of purely 

* The cases reported by Dr. Sanger Brown also resemble this type. 



392 THE NERVOUS DISEASES OF CHILDREN. 

spinal origin ; in other cases they were associated with dis- 
tinct cerebral symptoms. A classification of all of these 
various diseases according to the morbid conditions under- 
lying them, is not yet possible; it is sufficient for the pres- 
ent to discuss them from the etiological point of view. I 
have chosen, therefore, to designate them as hereditary 
spastic paralysis, and to subdivide them, according to their 
more prominent symptoms, into a spinal, and a cerebral or 
a cerebro-spinal variety. 

After adopting a subdivision of this kind, the pathology 
of each type will be more readily made out ; and it is quite 
probable that the various types will be found to represent 
a mere difference in the topographical distribution of le- 
sions and symptoms, while the morbid process, an arrest of 
development, or an early degeneration, is common to all. 

The spinal type of hereditary spastic paralysis is well 
illustrated by Newmark's cases. In view of the impor- 
tance of the subject a short summary of a few of his cases 
will be given. 



A girl, aged fifteen, tall and intelligent ; normal birth and labor ; no con- 
vulsions. Did not kick with the legs as other children do in bed. Began to 
walk at eighteen months. Gait peculiar from the first, and always remained 
typical of spastic paraplegia. Lower extremities flexed at the hips and 
knees ; adduction of thighs and pes equinus. No wasting of muscles, which 
react well to both currents. Knee-jerks and adductor reflex exaggerated ; 
no ankle clonus (probably on account of extreme contracture). No disturb- 
ance in the upper extremities, but all tendon reflexes exaggerated in them. 
Jaw-jerk present. Intelligence, sensations, and sphincters intact. No nys- 
tagmus, no strabismus. Girl is able to use a tricycle. Speech is normal. 
A brother, aged five, exhibits very similar symptoms. No history of syphilis 
in the family. There were no other cases of similar nature in the family, ex- 
cept one of spastic diplegia in a first cousin ; but this diplegia was evidently 
the result of difficult labor. 

In the second family the father is said to be a tall, healthy man of thirty- 
eight, who denies syphilitic infection or alcoholic habit. Has never known 
of any affection similar to that observed among his children. His reflexes 
are normal. Mother of children, aged thirty-seven, has always been healthy. 
Her knee-jerks and Achilles tendon reflex quite active. Lively reflexes in 
the upper extremities and jaw-jerk distinct ; no ankle clonus. No blood re- 
lationship between man and wife. They have had eleven children, eight 
of whom are living. The oldest living child is a boy, aged sixteen, who 
was well until about a year and a half before he was examined. At that 



HEREDITARY DISEASES OF THE SPINA I CORD. 2>93 

time a stiffness appeared in the legs, especially on rising in the morning. 
His condition gradually developed into a mild form of spastic paraplegia, 
with typical gait and position of the extremities. Knee-jerks much exag- 
gerated ; no patellar clonus nor ankle clonus. Plantar, cremasteric, and 
abdominal reflexes very lively. Abdominal muscles said to be rigid. Up- 
per limbs normal ; reflexes much increased. Jaw-jerk well marked. In a 
second brother, aged fourteen, the symptoms had become more pronounced, 
and had set in at the age of seven and a half years. The legs were stiff and 
walking was difficult ; had been compelled to use crutches. There were con- 
tractures in the knee-joints ; there was adductor spasm and pes equinus, 
furthermore, an exaggeration of the knee-jerks. Ankle clonus was present, 
and the plantar and cremasteric reflexes were also very lively. A third 
brother developed exactly the same symptoms at the age of nine years, after 
typho-malarial fever. Though these three cases are the most pronounced 
ones in the family, one sister exhibits increased reflexes ; a boy, eight years 
of age, is said to be stiff in the knees, with exaggeration of the patellar re- 
flex ; another child, aged six, is said to have a lazy, dragging walk, with ex- 
cessive increase of knee-jerks ; a child, aged three and one half, has active 
knee-jerk and jaw-jerk. 

That there is a tendency to spastic paralysis in this 
family there is no doubt ; but the fact that instruments were 
used at the birth of several children, including the one whose 
symptoms were most pronounced, makes it probable that 
" heredity " alone was not responsible for the multiplicity 
of cases. For the same reason Schultze's patients cannot be 
included under this heading; there was a history of difficult 
labor in all his cases. 

The symptoms of this spinal type (spastic paraplegia, 
with rigidity or contractures, increased reflexes) are 
distinctly due to interference with the pyramidal tracts 
in the lateral colunjns of the cord. Many years ago 
Striimpell described the cases of two brothers, who pre- 
sented the typical features of a spastic spinal palsy. In 
one of these cases a post-mortem examination was made, 
by which it was determined that the symptoms were due 
to a primary systematic degeneration of both pyramidal 
tracts in the lateral columns, together with a slight affec- 
tion of the cerebellar tracts and the columns of Goll. More 
recently Striimpell has described another case, very much 
like the other two, in which he made the diagnosis of he- 
reditary spastic palsy. In the family of this patient the 
grandfather was said to have suffered from a palsy of the 



394 THE NERVOUS DISEASES OF CHILDREN. 

legs, the father had a peculiar gait, and people who knew 
both the father and the present patient said that one walked 
like the other. Two uncles were said to have the same walk. 
The mother of the patient and other brothers and sisters 
were entirely healthy. The patient did military service 
from his twenty-first to his thirty-third year, and was not 
inconvenienced in any way, except that he occasionally 
noticed a peculiar feeling in the legs. At the age of about 
thirty-five his first symptoms began. His legs became stiff 
and he soon had to resort to a stick in walking. The exam- 
ination of the patient revealed a typical spinal spastic 
paralysis, with exaggeration of the reflexes, rigidity of the 
joints, normal sensation, and a spastic gait. The vesical and 
rectal reflexes were not interfered with. All the symptoms 
steadily increased, but no new ones were developed. In 
addition to its interest as a form of hereditary spinal disease 
the type also deserves some special recognition from the 
fact that, from first to last, the symptoms have been such 
as are due exclusively to disease of the lateral columns. 
Strumpell refers to Bernhardt's patients, and claims that 
they are entirely identical with those described by him. 
Four out of six brothers presented the phenomena of spastic 
spinal palsy ; three of these four developed their symptoms 
at the age of thirty, and all the morbid signs progressed in a 
remarkably slow fashion. I can agree with Strumpell in 
believing that these diseases are entirely similar to those de- 
scribed by him, although in one case of Bernhardt the symp- 
toms tended toward a cerebro-spinal rather than a purely 
spinal type. The forms described by Tooth and Philip 
should be placed in the same category. 

Spastic spinal paralysis (spastic paraplegia) occurs in children of families 
in which there is no history of a similar affection in the same or past genera- 
tions. In February, 1893, a girl, four years of age, was brought to my clinic, 
who was afflicted with a typical spastic paraplegia ; paresis and rigidity of 
both lower extremities ; very rigid gait, increased knee-jerks on both sides, 
• slight double ankle clonus — these reflexes being a little more marked on the 
right side than on the left side ; increased reflexes in the upper extremities, but 
no weakness. Electrical reaction of all muscles and nerves normal ; no dis- 
turbances of sensation ; no impairment of vesical and rectal reflexes ; no 
nystagmus. Intelligence good. The birth of the child (first-born) had been 
entirely normal and at full term ; no instruments were used ; the child was 



HEREDITARY DISEASES OF THE SPINAL CORD. 395 

supposed to be perfectly well, except that it did not begin to walk until it was 
three years old, and its walk was unusually stiff and awkward ; it dragged its 
feet. Three younger children are perfectly healthy. Excepting the early age 
of onset there is no distinction in clinical symptoms between such cases as 
these and the spastic spinal paralysis of the adult. 

It is best not to speculate regarding the morbid pathol- 
ogy of this type. The symptoms must be attributed to 
the pyramidal tracts of the lateral columns, but whether the 
disease in them is primary or secondary it is too early to 
state. It is probable that by disease, or by arrest of de- 
velopment, the entire pyramidal tract, and not merely the 
spinal portion of it, is involved. In view of Strumpell's inves- 
tigations there is no longer any doubt about the occurrence 
of a primary degeneration of the lateral columns develop- 
ing later in life, but due in all probability to abnormal con- 
genital conditions. There is some reason, therefore, to be- 
lieve that the question of the occurrence of a primary lateral 
sclerosis will be affirmatively answered through these stud- 
ies on hereditary spastic paralysis. 

Diagnosis. — The diagnosis of these diseases need not be 
given in further detail. The history of a case exhibiting 
the symptoms of spastic paraplegia of the lower extremities, 
with increase of the reflexes, with rigidities and contrac- 
tures, without involvement of the vesical or rectal reflexes, 
without atrophy, without disturbances of speech and nys- 
tagmus, would be sufficient to place the case in this cate- 
gory, provided the hereditary character of the affection 
could be established. The disease should be carefully dif- 
ferentiated from the spastic cerebral palsies of childhood.* 
The diplegias and paraplegias occurring as a result of dirh- 

* While this chapter was passing through the printer's hands, a reprint of Erb's ar- 
ticle on Hereditary Spastic Paralysis, reached me. Erb thinks that some of the 
congenital diplegias and paraplegias which Freud, myself, and others have attributed 
to cerebral lesions may at times be due to spinal lesions. I am willing to concede the 
possibility of this ; but I think that such spinal affections are, after all, rare ; and there is 
the one fact, to which I have alluded in various writings, that in forty-five per cent, of 
all the cases of infantile spastic paraplegia there is marked idiocy. This asso< 
militates against the diagnosis of a purely spinal disease. I have reviewed the his- 
tories of all my patients with supposed cerebral paraplegia andean find none which I 
am willing to attribute to a spinal lesion. The child reported on page 394, though suf- 
fering from paraplegia, was recognized as probably of spinal origin ; and such an 
origin may be suspected in types of spastic paraplegia, without idiocy coming on 
several years after a normal birth. 



396 THE XERVOUS DISEASES OF CHILDREN. 

culty during labor (^Little's disease), would be most easily 
confounded with this hereditary form. A careful inquiry 
into the history of labor, the determination of the exact age 
at which the trouble began, the frequent occurrence of con- 
vulsions and of defective mental development, in birth 
palsies, will help to differentiate between these two sets of 
cases. If spastic paraplegia is developed in a child, or in a 
youth, that has not been preceded by convulsions, or is not 
associated with defective mental development, the case 
would come more properly under the head of hereditary 
spastic paraplegia, and we must remember that such cases 
may occur without any history of similar disease in the same 
or preceding generations.* 

The prognosis of hereditary spastic spinal paralysis is 
a grave one as regards recovery from the disease, but not 
unfavorable as regards the duration of life, for the majority 
of patients thus far examined have been well advanced in 
years. In the way of treatment, nothing can be attempted 
except to apply the usual methods of massage and elec- 
tricity, or possibly to attempt to improve contractured 
limbs by various tenotomies. 

The Cerebral Type. — Cases of this character were de- 
scribed by Freud and myself several years ago in studies on 
the cerebral palsies of childhood. I am a little loath to re- 
move them from that category of diseases, but believe that 
they should be referred to in this connection for the sake of 
pointing out their exact clinical relation to the other forms 
mentioned in this chapter. 

Under the title of " Arrested Cerebral Development " I 
published my first case of this description in 1887, without 
suspecting the hereditary element; in 1892 I recorded the 
occurrence of a similar affection in a sister of my first 
case, and in that year was able to refer to another family 

* Marie and some oiher French authors deny the existence of a primary spinal 
spastic paralysis in the adult, and believe that if such a condition does exist, it is to be 
traced back to the early years of life. They describe as Tabes dorsal spasmodique of 
children, a group of symptoms which German and American authors have included un- 
der the term, Congenital Spastic Diplegia and Paraplegia. The French view would 
take all of these cases out of the category of cerebral diseases, and for this there is as 
yet no warrant. Marie (p. 101) states that "Tabes dorsal spasmodique" is never 
hereditary ; and attributes the condition to defective development of the pyramidal 
tract. This tract is surely a cerebro-spinal affair. 



HEREDITARY DISEASES OF THE SPINAL CORD. 



397 



in which four children were affected with this same dis- 
ease. 

The striking features of these cases are as follows: 
A child of apparently normal physical and cranial devel- 
opment thrives well until about the age of four or five 
months, then it begins to show marked retrogression ; does 
not take notice of things as other children do, cannot be 
induced to play ; does not recognize the nurse or mother. 
Its vision is defective and leads at an early day to blind- 




*ti 



Fig. 96. — Photograph of Brain of Author's First Case of the " Cerebral Type " of 
Hereditary Spastic Paralysis. The histological condition was described as an 
"agenesis corticalis. " In the above figure, the confluence of fissures and the ex- 
posure of the Island of Reil are the signs of a low order of cerebral development. 
Through the hardening process, the conditions have been accentuated a little, but 
they were present in the fresh specimen. At x, and in the frontal lobes, sections had 
been removed for histological examination. Other letters refer to fissures. 



ness. Nystagmus is present. The child utters a few 
sounds, but does not exhibit the least sign of intelligence. 
The symptoms continue in this way for a period of one year, 
or at the utmost two. The child's physical condition grad- 
ually deteriorates, and at the end of the specified period 
of time, after complete emaciation and marasmus, the child 
dies. Convulsions are never present. 

These cases appear to me to deserve special attention 



398 THE NERVOUS DISEASES OF CHILDREN. 

Among the large number of cases of idiocy that I have seen, 
and which I have attempted to analyze from a clinical and 
anatomical point of view, they stand out as a distinct group, 
and they are also distinct from the other spastic palsies. 
The disease has been noticed by ophthalmologists, and has 
recently been well described by Mr. Kingdon, of England, 
who has included among his list the report of one of my 
patients. 

My first case was examined by Dr. Knapp, when the child was about 
three months old. He reported vibratory nystagmus ; pupils contracted as is 
usual with children at this age. Media clear, optic-nerve discs pale. Fovea 
centralis of a cherry-red color, and surrounded by an intense grayish-white 
opacity. Dr. Knapp considered this condition to be due to a developmental 
defect ; ultimately there was complete optic atrophy. Discs as white as paper. 

In all of the children reported by various authors the fact of heredity has 
come out with unusual distinctness, several diseases of the same order occur- 
ring in successive generations, and in single families. The disease may be 
characterized as hereditary spastic paralysis, with idiocy and amaurosis. 

Freud has reported several interesting cases under the title of " Family 
Forms of Cerebral Diplegia." Two of the children were bright at the ages of 
six and five years respectively, but they presented from earliest childhood the 
following symptoms : Nystagmus, atrophy of the optic nerve, convergent 
strabismus, awkwardness in speech and in the use of the arms ; tremor of 
the arms and a spastic paraplegia of the lower extremities ; no convulsions. 
In these boys the disease is evidently present in its lightest and most favor- 
able form. The improvement in vision as the one child grew older seems 
evidence of this, but the resemblance of these forms to those described by 
me is shown by a third child that died at the age of ten months ; it was 
paralyzed from birth, and idiotic ; whether it was blind also is not stated. 
The father thought the child had died of rickets (more probably of maras- 
mus). 

Pelizaeus has described a family disease of which the chief symptoms 
were : Nystagmus, mental imbecility, disturbances of speech (Bradylalia), 
awkwardness of hands, spastic paraplegia of the lower extremities. The 
disease attacked male members of several generations, but was transmitted 
through healthy mothers. All of those who were attacked showed some 
symptoms in childhood, and several died young. Pelizaeus was inclined to 
the diagnosis of multiple sclerosis ; but his cases evidently constitute a late 
form of hereditary spastic paralysis.* 

The morbid anatomy of this cerebral type of hereditary 
spastic palsy is not fully known. In two of the children ob- 

* The cases of Home'n were probably of a syphilitic nature. The cases reported 
by Krafft-Ebing were instances of Little's disease, with a distinct history of premature 
delivery and other abnormal birth conditions. 



HEREDITARY DISEASES OF THE SPINAL CORD. 



399 



served by myself (two sisters), the chief changes consisted 
in a complete arrest in the development of the cortical cells, 
a condition to which the term agenesis corticalis seemed 
applicable.* The external configuration of the brain 
also proved it to be of a low order of development. 
Whether agenesis corticalis represents the entire morbid 
condition is doubtful, to say the least. Unfortunately I was 
not able to examine the spinal cord in either one of my 
cases. In all probability evidences of the arrest of devel- 
opment will be found in the cortex, in the pyramidal tracts, 
and in other parts of the central nervous system. (Fig. 96.) 




/ 





/ 



Cervical. Dorsal. 

Fig. 97. — Section through Cervical and Dorsal Segments in a Case of Meningo-En- 
cephalitis. Probable defective development of pyramidal tracts in the lateral col- 
umns ; no trace of a defect in the direct pyramidal tract. (Weigert stain.) 

These cases must be differentiated from the congenital 
diplegias (Little's disease), due to premature birth and ab- 
normal labor. This can *be done best by referring to the 
history of normal labor, to the absence of convulsions, and 
to the visual troubles. But a cerebral condition — a meningo- 
encephalitis — due to difficulties at birth, may be associated 
with a defective development of the pyramidal tract. This 
is the interpretation which I now give to the following case, 
which puzzled me for a long time, and presented a number 
of anomalous features.f 

The patient, a boy, one year of age at the time of death, was born of a 
healthy mother (primipara) after a dry labor of forty-eight hours. The child 

* For a fuller description of the anatomical condition, the reader is referred to the 
chapter on Diseases due to Defective Development of the Brain. 

tThis case was published in full in the New York Medical Journal for May a, [89] 






400 



THE NERVOUS DISEASES OF CHILDREN. 



was asphyxiated. From the first day to the age of six and a half months it 
passed through innumerable convulsive seizures of a very extreme order. (See 
Fig. 38.) It presented in addition : Retarded mental development ; spastic 



: t-._V;:-- - - "'■-, -^P '■;-." '—"' ' 



t^ 



■rr ♦^•T-^i- ■&*" 






3^<" 






f 

« 1 



yy^^' 




?-V«£'>. 




















B2 






a/ 



4A vfe 









■--4?-; 



Fig. 98.— Section through Motor Cortex in Case of Spastic Paraplegia, 0, space be- 
tween the cortex and pia ; b, blood-vessels in transverse and longitudinal sections, 
showing infiltration of their walls ; c, altered cells with pericellular spaces ; p, the pia, 
thickened and infiltrated, sending projection downward between two convolutions. 



paraplegia (upper extremities less paralyzed, but somewhat rigid) ; increased 
deep reflexes (patellar, clonus, etc.), and convergent strabismus. The epileptic 
seizures were lessened by treatment, but otherwise the condition remained 
unchanged. The child died of an acute infectious disease of a doubtful 
character. (So reported from the Babies' Hospital.) 



HEREDITARY DISEASES OE THE SPINAL CORD. 401 

On post-mortem examination I found distinct evidences 
of a wide-spread chronic meningo-encephalitis (probably due 
to meningeal hemorrhage at birth, see Fig. 98), and in ad- 
dition, what I supposed at the time was a secondary de- 
generation in the pyramidal tracts. But on re-examination 
of my specimens I was struck by the fact that there was 
relatively less sclerotic tissue in the diseased areas than in 
ordinary cases of secondary degeneration, and that the 
nerve-fibres were present in large numbers, but all of them 
were degenerated. The anterior pyramidal tracts, more- 
over, were not involved. (Fig. 97.) 

This degeneration or defect I now believe to have been 
primary and congenital A variation in the development 
of the pyramidal tracts in the lateral columns may account 
for the relative immunity of the upper extremities, al- 
though the cortical areas for both upper and lower ex- 
tremities were equally diseased. I do not wish to infer, 
however, that all cases of congenital spastic paraplegia are 
due to a spinal rather than a cerebral process. Some of 
them may be explained in this way ; but I cannot adopt 
the views of Marie and other French authors until further 
evidence of a purely spinal origin shall have been furnished 
in a number of cases. 

In all cases of hereditary spastic paralysis the prog- 
nosis is grave as regards recovery, or normal development 
of mind and body ; the probable duration of life can be 
gauged by the severity of the symptoms. The cases asso- 
ciated with idiocy and blindness die between the ages of 
one and three years. Treatment is purely symptomatic. 

As a preliminary division of the diseases discussed in 
this chapter, the following is submitted : 

HEREDITARY AND FAMILY AFFECTIONS. 

. . j i. Friedreich's Disease. 

Hereditary Ataxy, -j ^ Hereditary Cer ebellar Ataxy (Type, Nonne-Marie). 

f Spinal Type. Cases of Newmark, Bernhardt. Strum- 
Hereditary Spastic 1 pell, Erb, Tooth, and others. 

Paralysis, ) Cerebral or Cerebro-spinal Type. Cases of Pelizaeus, 

[ Sachs, Freud. 
Hereditary Progressive Muscular Atrophy.* 

* (Described in the following chapter. See page 409. ) 
26 



402 THE NERVOUS DISEASES 0E CHILDREN, 

BIBLIOGRAPHY. 

FRIEDREICH'S DISEASE. 

Besold : Deutsche Zeitschr. f. Nervenheilkunde, Bd. V., p. 157. 

Brown, Sanger : Brain, vol. xv., p. 250. 

Bury, Judson S. : Brain, July, 1886, vol. ix., p. 145 (full literature). 

Dejerine et Letulle : Semaine Medicale, March 12, 1890. 

Erb : Neurologisches Centralblatt, 1890, No. 12, p. 378. 

Friedreich : Virchow's Archiv, Bd. LXVIII. and Bd. LXX. ; also Arch. f. 

Psych., Bd. VII., 1876. 
Griffith : Transactions of College of Physicians, Philadelphia, 1888. 
Hammond : Journal of Nervous and Mental Disease, 1882. 
Kahler u. Pick : Arch. f. Psych., Bd. VIII., 1878. 
Ladame : Brain, vol. xii., p. 467. 
Marie : Maladies de la Moelle, p. 381, 1892. 
Ormerod : Brain, vol. vii., p. 105 ; vol. xv., p. 268. 
Remak : Berl. kl. Wochenschr., 1885, No. 7. 

Rook and Dana : Journal of Nervous and Mental Disease, 1890, p. 173. 
Schultze, F. : Zeitschr. f. Nervenheilkunde, Bd. V. ; Virch. Arch., Bd. 

LXXIX. 
Seeligmuller : Arch. f. Psychiatric Bd. X., 1880, October, 1885. 
Seguin, E. C. : New York Medical Record, 1885. 
Senator : Berl. kl. Wochenschr., 1893, No. 21. 
Smith, W. E. : Boston Medical and Surgical Journal, 1885. 

HEREDITARY CEREBELLAR ATAXY. 

Brissaudet Londe : Revue Neurologique, No. 5, March 15, 1894. 

Marie: Semaine Medicale, 1893, p. 444. 

Menzel: Hereditary Ataxy and Cerebellar Atrophy, Arch. f. Psych., Bd. 

XXII., p. 160. 
Nonne: Arch. f. Psych., Bd. XXII. 

HEREDITARY SPASTIC PARALYSIS. 

Bernhardt: Virchow's Arch., Bd. CXXVI., 1891. 

Bloch: Archiv f. Psychiatric Bd. XII. 

Erb : Zeitschr. f. Nervenheilkunde, Bd. VI., December, 1894. 

Freud: Zur Kenntniss der Cerebralen Diplegien. Leipzig u. Wein, 1893, 

p. 143; also, Neurolog. Centralbl, 1893, Nos. 15 and 16. 
Homen : Arch. f. Psych., 1892, Bd. XXIV., p. 191. 
Krafft-Ebing : Wiener kl. Wochenschr., 1892, No. 47. 
Marie : Les Maladies de la Moelle. Paris, 1892, pp. 87-107, 
Pelizaeus : Arch. f. Psychiatric Bd. XVI. , p. 698. 
Philip : Brain, vol. viii. } 1886. 



HEREDITARY DISEASES OE THE SPINAL CORD. 403 

Sachs : Journal of Nervous and Mental Disease, 1887, p. 541 ; 1892, p. 603 ; 

Volkman's Vortag, Nos. 46 and 47, p. 467. 
Seeligmiiller : Deutsche Med. Wochenschrift, 1876, Nos. 16 and 17, Arch. f. 

Psychiatrie, Bd. X., p. 222, 1880. 
Striimpell : Arch. f. Psych., Bd. XVII. ; Deutsche Zeitschr. f. Nervenheil- 

kunde, Bd. IV. 
Tooth : St. Bartholomew's Hospital Reports, xxvii. ; see also Neurolog. Cen- 

tralbl., 1802, No. 8. 






CHAPTER XXIII. 

PROGRESSIVE MUSCULAR ATROPHIES. 

In this chapter we intend to discuss all those diseases 
which are characterized by a progressive weakness and 
atrophy of certain groups of muscles. We have nothing 
to do with muscular atrophy, whether progressive or not, 
which follows after acute disease of the brain, of the spinal 
cord, or of the peripheral nerves. The term "progressive 
muscular atrophy " was formerly given to a single type of 
disease with which we shall become more intimately ac- 
quainted, and for a time all cases resembling this one type 
were designated in the same way. It became evident, how- 
ever, that this one term was altogether too general. While 
it was a convenient clinical designation for an entire group 
of diseases, it did not sufficiently describe a number of 
other forms which were closely allied to the chief type. 
In order to avoid confusion, it would be well if we could 
dismiss the term progressive muscular atrophy altogether, 
for in many of the cases hypertrophy as well as atrophy is 
present for a long period of time. The word dystrophy, 
which might be used to designate both conditions, has, un- 
fortunately, been restricted to the cases of primary or idio- 
pathic muscular wasting ; we cannot, therefore, apply it to 
the spinal forms. Moreover, a number of diseases were 
included under the heading of progressive muscular atrophy 
which we now recognize to have been cases of amyotrophic 
lateral sclerosis, of syringomyelia, and possibly of spinal 
syphilis. 

The chief question at the present time regarding the 
various forms of progressive muscular atrophy is, whether 
in a given case the disease is of spinal or muscular origin. 



PROGRESSIVE MUSCULAR ATROPHIES. 



405 



Amyotrophy . 



Neural Atrophy 



Those forms of progressive muscular atrophy due to a 
spinal lesion we call amyotrophies, and to those due to disease 
of the muscular system only we give the name myopathies. 
We shall see that there is some reason, too, to constitute a 
third type, which we might designate as a neural form of 
progressive muscular atrophy. (Fig. 99.) 

The study of this entire subject began many years ago 
with the establishment of two distinct diseases — the first was 
the typical " progressive muscu- 
lar atrophy," as described by 
Aran and Duchenne ; the sec- 
ond, pseudo-hypertrophic mus- 
cular paralysis. Since that 
time at least six different forms 
of progressive muscular wast- 
ing have been described, and 
the attempt has been made in 
each case to prove the relation 
of the special form, either to 
the Aran-Duchenne type, or to 
the type of muscular pseudo- 
hypertrophy. The Aran-Du- 
chenne type has become the 
chief exponent of progressive 
amyotrophies ; while muscular 
pseudo-hypertrophy has been 
considered the most pro- 
nounced form of primary myo- 
pathies. The various types of 
progressive muscular disease 
have been established very largely in accordance with 
the mere topographical distribution of atrophy or hyper- 
trophy. Though convenient for clinical designation, such 
a distinction is not sufficient for a rational classification 
of these various diseases. It must be our aim to find the 
cardinal symptoms which will help us to differentiate at 
once, and easily, between those cases of progressive mus- 
cular disease due to spinal-cord lesions and those primary 
dystrophies, which represent a disease of the muscular 
system. 




Myopathy 



Fig. 99. — A Diagram designed to show 
the Site of the Morbid Lesion in the 
Several Groups of Progressive Mus- 
cular Atrophy. 



406 THE NERVOUS DISEASES OF CHILDREN. 

The following are the cardinal symptoms present in the majority of cases 
belonging to the two large groups of cases : 

Progressive Amyotrophies. Progressive Myopathies. 

Onset late in life ; rarely in early Onset in early life. 

childhood. 

Not hereditary, as a rule. Generally hereditary (family trouble). 

Wasting first in the upper extremi- Wasting or hypertrophy begins in 

ties (leg type rare). the lower extremities. 

Hypertrophy does not occur. Hypertrophy frequent. 

Fibrillary twitchings. No fibrillary twitchings. 

Reaction of degeneration often pres- Reaction of degeneration rare (quan- 

ent in affected muscles. titative, not qualitative, electrical 

changes). 

These points of differential diagnosis will hold good in the majority of 
cases. But cases of spinal progressive muscular disease occur in which there 
is a strong history of an hereditary or family affection. Fibrillary twitch- 
ing has been seen in some cases that appeared to be typical myopathies, 
and the electrical reactions have been found considerably altered in similar 
cases, so that of all these cardinal symptoms there are, after all, only a very 
few which are invariably present in one or the other form of progressive mus- 
cular disease, and it is wiser, therefore, to be guided by the general agree- 
ment of symptoms rather than by any one single symptom.* This confusion 
of types of disease and of symptoms, need not cause surprise, if we remem- 
ber that the ganglion cells of the spinal cord, the peripheral nerves, and the 
muscles, constitute a physiological unit (Neuron). 

With these prefatory remarks we may proceed to the 
consideration of that class of cases which for a very long- 
time were supposed to be the only representatives of what 
was formerly called "progressive muscular atrophy." This 
is a spinal-cord affection which, as a rule, begins late in life. 
It might, therefore, be considered out of place to treat of 
this disease in a work on the nervous diseases of children ; 
but the entire subject of muscular diseases cannot be prop- 
erly understood unless we can recognize this special type, 
and, furthermore, cases of this type have of late years been 
described by Hoffmann and others, in children. 

* The hereditary cases reported by Werdnig are particularly interesting in this re- 
spect. 



PROGRESSIVE MUSCULAR ATROPHIES. 407 

PROGRESSIVE AMYOTROPHY. — " PROGRESSIVE MUSCULAR 
ATROPHY." (TYPE, ARAN-DUCHENNE.) 

This form begins, in the majority of the cases, with an 
atrophy and a corresponding weakness in the small muscles 
of the hand (thenar and hypothenar). The atrophy extends 
slowly from muscle to muscle (" atrophie individuelle "), be- 
ginning as a rule with the adductor pollicis. It involves by 
degrees the opponens pollicis and the deep muscles of the 
thenar. From these it gradually extends to the muscles of 
the hypothenar, the interossei, the flexors and extensors in 
the forearm. At this point the disease may remain sta- 
tionary, or it may spread to the flexors in the upper arm, to 
the deltoid, the triceps, and finally to the muscles of the 
trunk, the shoulders, and the back. Duchenne recognized 
the fact that the atrophy may, in exceptional cases, begin in 
the trunk, in the shoulders, or in the legs. Some of these 
would now, no doubt, be considered under a different head- 
ing, but I have myself seen cases which could in nowise 
be distinguished from the typical Aran-Duchenne disease 
which began in the muscles of the thighs. If the disease 
begins in the upper extremities, the legs are, as a rule, not 
affected until very late in the course of the disease ; but 
there are exceptions to this rule. I have observed several 
cases in which the atrophy in the lower extremities began 
almost simultaneously with that in the upper extremities. 

The atrophied muscles in this form exhibit fibrillary con- 
tractions, and, as a rule, present marked changes in electri- 
cal contractility. These changes are not so pronounced as 
in cases of acute or subacute anterior poliomyelitis, and yet 
after the disease has lasted for a considerable length of time 
a typical reaction of degeneration will be found present in 
most of the wasted muscles. In the majority of cases thus 
far recorded the disease has not been distinctly hereditary, 
although series of such cases with distinct hereditary ten- 
dencies have been published by Naunyn, Eichhorst, Ham- 
mond, Osier, and others * 

* The famous Weathersby family, reported by Hammond, and the Fair family, of 
Vermont, described by Osier, may possibly represent other types of progressive 
muscular atrophy, which we shall consider later on. 



408 THE NERVOUS DISEASES OF CHILDREN. 

As the disease progresses the wasting becomes more and 
more extreme ; the patient is no longer able to get about, 
becomes bedridden, and after mairy years of annoyance, if 
not of suffering, dies of some intercurrent disease, or from 
extension of the process upward into the region of the me- 
dulla, with consequent paralysis of the vital centres. 

The symptoms of the disease were well described by 
Duchenne, and very little has been added since his day to 
the clinical characterization of this special form. But a very 
warm discussion was waged for a long time regarding the 
origin of the disease, some maintaining its spinal origin, 
others believing it to be a peripheral disease. There was 
some reason for this difference of opinion, for the cases upon 
which the older authors based their views were in part due 
to spinal disease, and in part due to nerve or muscular 
lesions. The microscopical studies of Charcot and Joffroy, 
of Lockhart Clarke, of Hayem, and others, proved beyond 
a doubt that this special form of progressive muscular 
atrophy was due to changes in the spinal cord. The chief 
changes found are these : A sclerotic and pigmentary atro- 
phy of the ganglion cells of the anterior horns, inflamma- 
tory changes in the neuroglia, increased size of the blood- 
vessels, and proliferation of the cellular elements. In fresh 
preparations granular corpuscles are found, and according 
to the degree and stage of the disease the anterior horns 
may be very much reduced in all diameters, and the gan- 
glion cells either atrophied or entirely lost. The anterior 
nerve-roots are affected secondarily to the lesion of the gray 
substance. The nerve-fibres are not all destroyed, a num- 
ber of them remaining intact. Those that are destroyed 
exhibit the appearances of simple atrophy, a point to which 
Charcot alludes as distinguishing these cases from infantile 
spinal paralysis. 

According to these pathological findings we must sup- 
pose that an inflammation spreads slowly from the ganglion 
cells of the anterior horns along the anterior nerve-roots 
without destroying as many of these fibres as is the case in 
infantile poliomyelitis. The atrophic changes in the mus- 
cles are the direct result of irritation, which begins in the 
cells of the anterior horns, and is propagated thence through 



PROGRESSIVE MUSCULAR ATROPHIES. 4O9 

normal or only half-wasted nerve-roots, to the peripheral 
muscular fibre. Positive as these anatomical findings seem 
to be, it is somewhat surprising to learn how few reliable 
post-mortem examinations have been made in these cases 
proving the correctness of these views. The cases of Pier- 
rot -Troissier, of Striimpell and of Hoffmann, are among 
the few which have been so carefully examined as to have 
placed the spinal origin of this special type of progressive 
muscular atrophy beyond question. In these cases the an- 
terior gray matter was the only part affected, and alone re- 
sponsible for the wide-spread muscular atrophy. 

There is no need in this treatise to give a typical history 
of a case of spinal progressive muscular atrophy, as it oc- 
curs in the adult. I have stated above that these cases are, 
as a rule, not hereditary, and upon this absence of the factor 
of inheritance the differential diagnosis was formerly fre- 
quently based between a progressive amyotrophy and a pro- 
gressive myopathy. But this point of differential diagnosis 
has been rudely shaken by the interesting article which 
Hoffmann published only recently concerning the occur- 
rence of chronic spinal muscular atrophy in children on an 
hereditary basis. I will endeavor to summarize one of his 
cases of 

HEREDITARY PROGRESSIVE MUSCULAR ATROPHY. (HOFFMANN.) 

A girl, four years of age ; the birth of the child was entirely normal ; 
when nine months of age was able to stand ; early abnormal development 
of adipose tissue. Gradually the child became so weak that it could not 
stand, could not sit upright in bed, could not turn around without assistance. 
For a long time it was able to move its feet and its arms. The motor dis- 
turbances increased gradually, and the child lost its superfluous fat and be- 
came thoroughly emaciated, particularly in the trunk and the extremities. 
The face remained full. The sphincters were at no time involved. Mental 
development was good. Speech was normal ; no convulsions ; no strabismus ; 
no difficulties in mastication or deglutition. The child was able to turn its 
head, but could not lift it from the pillow. There was no evidence of any 
hypertrophy or pseudo-hypertrophy in any of the muscles, but there was pa- 
resis and atrophy of the deep muscles of the neck, of the sterno-eleido-mas- 
toid, of the trapezius, of most of the shoulder-muscles, of the latissimus dorsi, 
of the serrati, the pectoral and deltoids, and of the flexor muscles in the arm. 
The biceps and brachialis anticus were very thin, the supinator longus a little 
stronger than these. The triceps was thin and weak. The extensors and 



410 THE NERVOUS DISEASES OF CHILDREN. 

flexors in the forearm were also atrophic and paretic. The thenar and hypo- 
thenar eminences were thin, flaccid, and weak, as were also the individual in- 
terossei. The paralysis was in proportion to the atrophy of these muscles. 
The tendon reflexes were entirely wanting in the upper extremities. The 
mechanical excitability of the muscles was diminished. The nerve-trunks 
were neither thickened nor sensitive on pressure. The paresis and atrophy 
of both upper extremities were entirely symmetrical. There were no fibrillary 
movements. There were no trophic or vasomotor disturbances of the skin 
in the upper extremities. Sensation was entirely normal. There was marked 
diminution of electrical excitability in the median and ulnar nerves, and com- 
plete reaction of degeneration in the biceps. 

The muscles of the back and abdomen were very paretic, the long mus- 
cles of the spine much diminished in volume and power. There was lor- 
dosis of the lumbar region of the spinal column. The gluteal muscles, and 
all the muscles of the thigh, were very atrophic and almost completely para- 
lyzed. The muscles of the leg were also atrophic and paretic. Movements 
of the toes tolerably good. A progressive diminution in the volume of the 
calf and thigh muscles was noted at periods six months apart. No indica- 
tion of any deep reflexes in the lower extremities. The faradic excitability 
of the nerves in the lower extremities was distinctly diminished. No sensory 
disturbances, no fibrillary or fascicular or choreic movements of muscles. 
The paralysis was symmetrical and flaccid. The joints were like those of 
infantile spinal palsy. The child died of an intercurrent pulmonary trouble 
about one year after the first examination. 

The report of the autopsy included the following points : The lumbar 
portion of the spinal cord less in volume than under normal conditions. 
Very marked atrophy of the anterior spinal-cord roots throughout the entire 
spinal cord as high up as the medulla. On microscopical examination the 
chief changes found were in the anterior gray matter and in the anterior 
nerve-roots, from the lower portion of the medulla through the whole spinal 
cord. There was a distinct atrophy and diminution in number of the gan- 
glion cells of the anterior horns throughout the entire spinal cord. This was 
more marked in the lumbar than in the cervical portion. There was also 
very marked atrophy of the anterior roots and a similar affection in the 
peripheral nerves and the nerve-filaments in the muscles, as well as a very 
marked atrophy of the muscles supplied by these nerves. In addition to 
these chief changes there were also slight and symmetrical changes in 
the motor tracts of the spinal cord, particularly in the crossed pyramidal 
tracts and in the lateral columns as well as in the direct pyramidal tracts. 
Medulla oblongata was not involved. 

This case is so similar, from a clinical and anatomical point of view, to 
the typical cases of the Aran-Duchenne type that the close relationship be- 
tween these forms cannot be doubted. It remains to add that an entirely 
similar affection was reported in the case of a brother of the first child, in 
whom the disease began at about the same age and behaved in very much 
the same way. The parents of these two children have raised a family of 
fifteen ; several of these have died of convulsions, others are afflicted with 



PROGRESSIVE MUSCULAR ATROPHIES. 4 1 I 

distinct lipomatosis, and the parents invariably accepted the occurrence of 
this excessive accumulation of fat as an evil omen. 

Abortive and hereditary forms of progressive muscular atrophy of the 
spinal type have come under my notice. The cases are those of a physician, 
living in Canada, and his daughter, about twelve years of age ; in both of 
them there was distinct atrophy of the interossei of both hands, flattening of 
the thenar, and slight extension of the atrophy to muscles of the forearm. 
" Weak hands " have been characteristic of the family, but the disease does 
not appear seriously to invade other parts. The father (physician) has had 
this trouble for years, and is now compelled to give up surgical work. 



PROGRESSIVE NEURAL MUSCULAR ATROPHY — PROGRESSIVE 
NEUROTIC MUSCULAR ATROPHY — THE PERONEAL FORM, 
OR LEG TYPE, OF PROGRESSIVE MUSCULAR ATROPHY.* 

In this form of progressive muscular wasting the dis- 
ease begins, in the majority of cases, in the lower extrem- 
ities. At first the extensor muscles of the toes show a 
slight weakness. The small muscles of the feet may be- 
come involved, and then the atrophy spreads very much 
after the fashion of the spreading of the atrophy in the 
spinal cases, from muscle to muscle, until the entire leg is 
considerably atrophied and weakened. As a result of this 
weakening, deformities of the foot may arise ; pes equinus 
or pes equino-varus is a frequent result. In other cases a 
distinct club-foot is developed, and inasmuch as the affec- 
tion may spread quite rapidly from one side to the other, 
a progressive form of wasting in both lower extremities, 
including possibly the development of double club-foot, is 
extremely suggestive of this " leg type " of progressive 
muscular atrophy. The disease, as Hoffmann has shown, in 
rare instances attacks the upper extremities first and then 
involves the lower. Hoffmann has objected to the use of 
the designation " leg type," but since in this form the 
legs are involved at a very early stage, whether the disease 
begins in them or in the upper extremities, it seems proper 
for the present to retain this designation. The atrophy in 
the upper extremities may involve the small muscles of the 
hand, the extensor and flexor muscles of the forearm or the 

* This disease is also known as the type of Charcot-Marie-Tooth. Hoffmann sug- 
gested the term "progressive neurotic," Bernhardt, the term " progressive neural,'* 
muscular atrophy. 






412 THE NERVOUS DISEASES OF CHILDREN. 

arm, and may cause a wasting of the muscles about the 
shoulder-girdle. I have seen the infra- and supra-spinati 
especially wasted in several of these cases. The atrophy 
in the upper extremities is, as a rule, not so distinct nor so 
early a symptom as in the cases of the Aran-Duchenne type. 




Fig. ioo. — Two Brothers afflicted with the Peroneal Form of Progressive Muscular 
Atrophy, Eight Months and One Year respectively after First Operation. 

Sensory changes are generally present, and serve as an 
important point of differentiation between this special form 
of atrophy and a spinal amyotrophy. The various forms 
of sensation may be slightly altered, or, in some cases, tac- 
tile sensation and temperature sense may remain normal, 
while the pain sense may be more distinctly involved. Par- 
amnesias may be present in addition to the objective 
changes in sensation. The reflexes in the lower extremities 
are either diminished or lost ; the exact state of the reflexes 



PROGRESSIVE MUSCULAR ATROPHIES. 413 

depending somewhat upon the stage of the disease at the 
time the patient is examined. The electrical reactions in 
the atrophied muscles are, as a rule, altered. The changes 
are not so extreme as in the cases of spinal amyotrophy, 
nor are they as mild as in the primary muscular dystrophies. 
The reactions are diminished quantitatively, and altered 
also as regards the quality of contractions. A case has not 
yet been reported in which the muscles of the face were 
involved, and there were no changes in sensation, and none 
in electrical reactions in any of the muscles or nerves 
of the neck and head. 

The symptomatology of this rare form will be best elucidated by an 
extract from the account given of the disease as it occurred in two brothers 
which was reported in a paper published by the author in the year 1890. 
These cases are all the more interesting as they are the only ones, to my 
knowledge, which for a time were successfully treated by surgical measures. 

The family history is very meagre. The father, a Bavarian, is dead ; 
cause of death unknown. Mother, living and healthy, thirty- two years of 
age. The two patients were the only children. Both boys were born healthy. 
Each showed disturbances in the use of the legs at a very early day, and at 
the age of five years both had acquired double club-foot. 

When the younger brother was first admitted to the hospital, in 1887, he 
was compelled to use crutches. According to the hospital records there 
was marked shortening of the Achilles tendon and plantar fascia of both feet. 
Foot arched (pes cavus) ; when at rest inner side does not touch the floor. 

Measurements : Right calf, 7 inches ; left calf, 7 inches. Dr. Gibney 
performed double achillotomy. Separation of ends ij£ inch on the left 
and almost the same on the right side. Feet were flexed dorsally to about 
eighty degrees, and plaster-of-Paris splints were supplied. He was dis- 
charged four months after the operation, with a note that the patient walks 
quite well, soles flat on the ground, toes slightly inverted. He returned to 
the hospital in 1888 with paralytic limp and with a position of the feet as rep- 
resented in the accompanying figure. Double achillotomy was again per- 
formed, division of plantar fascia? was made, and there followed application 
of Thomas's tarsoclast and plaster splints with the results as shown in Figs. 
101 and 102. Two months after the operation the feet were in typical cal- 
caneus position, when using his shoes without apparatus ; standing squarely 
on the soles of the feet he shows disposition to roll feet inward. He could 
voluntarily flex the ankle-joint a little beyond ninety degrees, but in so doing 
the toes were hyperextended. He walks very much as children do with a 
peroneal type of poliomyelitis. Marked disposition to pes varus. 

My own examination elicited the following points : The 
boy was of stouter and shorter stature than his brother. 



4H 



THE NERVOUS DISEASES OF CHILDREN. 



Intelligence good. His broad chest and fat stomach are in 
curious contrast to his spindle-shaped extremities. Circum- 
ference of chest, 26 inches. Right arm, 6^ inches ; left arm, 
63^ inches. Right forearm, 6^ inches ; left forearm, 6]^ 
inches. Grasp of both hands very weak. A general emaci- 
ation of all parts of upper ex- 
tremities. Very distinct atro- 
phy of infraspinatus. In the 
legs general atrophy is very 
well marked. The right 
thigh, four inches above pa- 
tella, 1 1 inches ; left thigh, 
10^ inches. Right calf, at^ 
greatest circumference, 8 
inches ; left calf, 8^ inches. 
The boy walks with a slightly 
waddling gait and has great 
difficulty in climbing the 
stairs. He can raise toes slight- 
ly on the left side, less well 
on the right side. Can raise 
left leg on tiptoe, but cannot 
do this with the right leg. In 
attempting to raise the whole 
body on tiptoe, falls forward. 
Sensation : Tactile sensation 
normal as determined by cot- 
ton, pin test, and the writing 
of numbers on the skin. Tem- 
perature sense normal. Pain 
sense exaggerated. Muscu- 
lar sense normal. Plantar re- 
flexes present and knee-jerk about normal. Slight lividity 
of legs, not so marked, however, as in the case of his 
brother. 




Fig. ioi. 
(Same patient as in Fig. 102.) 



The electrical examination : In the upper extremities the faradic response 
in the median and ulnar nerves was decidedly diminished. In the median 
nerve first KCC with 13 MA; ACC not at 20 MA. Galvanic current: In 
the right leg no reactions could be obtained by excitation of the nerves with 
currents used. In the extensor hallucis longus the first KCC and ACC were 



PROGRESSIVE MUSCULAR ATROPHIES. 



415 



obtained with a current of 14 MA. The tibialis anticus did not respond to 
currents of 20 MA. The anterior thigh muscles and posterior thigh muscles 
respond to strong currents of 16 MA, without reversal of formula. In the 
Jeft leg, the extensor hallucis, first KCC with 16 MA ; first ACC with 18 
MA. No contractions could be obtained by direct excitation of the tibialis 
anticus, with currents up to 20 MA ; on excitation of the extensor digitorum 
communis there is a slight movement 
of the small toe. 

The electrical examination, there- 
fore, shows that the reaction of de- 
generation is present in its typical 
form in most of the muscles below 
the patella, the galvanic excitability 
of the peroneal nerve being entirely 
lost. It also shows changes in elec- 
trical behavior in nerves of the upper 
extremities, since the responses of 
the ulnar and median nerves were 
markedly diminished. 

Comparing the histories 
of the two brothers, it was 
noted that they resemble 
each other very closely as 
regards the first appearance 
of the symptoms and the 
manner in Avhich the disease 
spread from muscle to mus- 
cle ; but there were also 
certain differences, such as 
the more marked electrical 
changes in the younger 
brother and the greater in- 
volvement of the upper ex- 
tremities in him, than in the 
older boy. In the younger 
brother the disease was more 
fully developed in every re- 
spect than in his older broth- 
er ; but such variations as occurred were within a reason- 
able limit and will serve to show to what extent variations 
may occur in persons undoubtedly suffering from the same 
type of disease. 




Fig. 102. — The Vounger of the Two 
Brothers (see Fig. 100) after Second 
Operation, showing Correction of De- 
formity of Feet, Marked Atrophy of 
Legs, and Incipient Atrophy of Muscles 
above Elbow and around the Shoulder 
Girdle. 



41 6 THE NERVOUS DISEASES OF CHILDREN. 

In addition to the symptoms which these boys have exhibited, it is inter- 
esting to note that Vizioli has reported the occurrence of amaurosis in a 
similar case, due to an optic-nerve atrophy. This symptom would support 
the argument in favor of the nerve origin of the disease. Furthermore we 
may insist on the fact that hypertrophy has not been recorded in any case, 
and that fibrillary movements seem to occur in some. 

Thomson and Bruce have reported an interesting case of a progressive 
muscular atrophy in a child ; but they nave not attempted to classify it under 
any special type. The disease began in the lower extremities, and gradually 
extended to the upper, involving both upper extremities and the muscles 
of the back. The gradual changes are well represented in Figs. 103-105. 
The child exhibited some disturbances of sensation (hyperalgesia) ; marked 
paresis in neck, back, and abdomen — most marked in loins, buttocks, and 
legs ; least marked in shoulders and arms ; no hypertrophy of any muscles. 
Electrical reactions at first little altered ; later on faradic excitability consid- 
erably impaired ; more markedly in the legs than in the arms. The report 
of the galvanic responses is too uncertain to permit of positive inferences ; the 
disease was steadily progressive. The case is all the more unique, as the 
authors found a spinal lesion and only very slight changes in the peripheral 
nerves.* 

Etiology. — Nothing more need be said upon this head 
than may be inferred from the previous histories. The 
disease is evidently a family affection, sometimes beginning 
at a very early age, as in my own cases, or appearing as 
late as the age of twenty, as in the one case described by 
Charcot-Marie. Whether the cases described by Osier as 
occurring in the Farr family of Vermont, which set in as 
late as the age of forty-six in some of the subjects, belong 
to this category or not is questionable, as the cases were 
reported before this special type of progressive muscular 
atrophy was known. In both my cases a thorough drench- 
ing of the skin by exposure to wet was mentioned, but I can- 
not attribute any further importance to this fact than that 
it may have helped to hasten a disease which was latent in 
the systems of the boys. 

Diagnosis. — The diagnosis of this "leg type," or neural 
form, of progressive muscular atrophy rests upon the recog- 
nition of the early beginning of the disease and of its hered- 
itary or family character. Moreover, the paralysis begin- 
ning in the leg muscles, and spreading to the upper extrem- 

*The case appears to bear a close resemblance to the peroneal form, as well as to 
an hereditary spinal form. 



PROGRESSIVE MUSCULAR ATROPHIES. 



417 







- 3 "-^^^Bi 








■P$ ; 


*M|; 


p& ; 
















v*'M" 


i%>" ■ ? 




^li 


mm? s/ 


MM 


P^, ,^^*** 








1 ^%^ * 


-***||g^'*\ 




^fe^ 








ta^..... 1 


Hi 



"3 S-. 

Pi tH) 




o <u 

p 

rl P 




O G 



£ £ 



27 



41 8 THE NERVOUS DISEASES OF CHILDREN. 

ities is associated with slight changes in sensation in the 
legs as well as in the arms. The occurrence of double 
club-foot (not congenital) will help to make the diagnosis 
still more certain. 

The disease will have to be differentiated from hereditary ataxia, from 
chronic multiple neuritis, from poliomyelitis, and from the primary muscular 
dystrophies. 

From hereditary ataxia the disease can be readily distinguished by the 
absence of the peculiar unsteadiness in walking and standing, by normal 
electrical reactions in cases of hereditary ataxia, and by the persistence of the 
reflexes in many of the cases of the peroneal form. 

From chronic multiple neuritis we can distinguish the " leg type " by the 
fact that pain plays an even greater role in most cases of neuritis ; that the 
atrophy is not steadily progressive, and that neuritis, of however long stand- 
ing, rarely leads to double club-foot ; and furthermore, that neuritis is not apt 
to occur as a family affection. 

From poliomyelitis we can differentiate these cases by the very gradual 
development of the disease, in contrast to the more sudden onset in infantile 
spinal paralysis ; by the progressive development of the atrophy, in contra- 
distinction to the retrogressive character of the wasting in poliomyelitis. 
Poliomyelitis is not an hereditary or family disease, and if the wasting in a 
case of poliomyelitis is as great as in cases of the leg type of progressive 
muscular atrophy the knee-jerks and other deep reflexes will surely be lost, 
while in the leg type they may be preserved for a considerable period of time. 
It is not so easy always to distinguish these cases from the subacute forms of 
poliomyelitis ; but if there is any doubt about the diagnosis in the earlier stages 
of the disease, the further progress of the trouble will remove all uncertainty. 

The disease might also be confounded, in its later stages, with the Aran- 
Duchenne type of progressive muscular atrophy, particularly if, as sometimes 
happens, the atrophy in those cases attacks the legs very soon after it has be- 
gun in the upper extremities. Under such conditions we must rely for the 
differential diagnosis upon the fact that the Aran-Duchenne type begins, as 
a rule, much later in life ; that it is rarely of an hereditary character, and 
that sensation is never affected in those cases as it is in the cases now under 
consideration. But I must concede that the clinical resemblance may be so 
strong between these two forms of disease that it will be practically impossi- 
ble to differentiate between them. 

Pathology. — For a number of years after this disease 
was first described the morbid anatomy was based upon 
mere speculation. Reference was made by several authors, 
among them by Hoffmann, to the older records of post-mor- 
tem examination by Virchow, Friedreich, and others. In 
these cases the authors found a degeneration of the nerves 






PROGRESSIVE MUSCULAR ATROPHIES. 419 

and a degeneration of the columns of Goll, but no satis- 
factory statement could be made at that time, which ante- 
dated the discovery of modern staining methods, regarding 
the disease of the anterior ganglion cells. I objected to 
the use of these older records as a proof of the non-involve- 
ment of the spinal cord, but all doubt regarding the origin 
of at least some of these cases has been removed by the re- 
cent studies of Dubreuilh (Revue de Medecine, 1890, p. 441) 
(n a very typical case of a child dying, in a family of which 
the mother and eleven children were affected with the same 
trouble. Dubreuilh proves that there were old changes in 
the peripheral nerves, particularly in the motor nerves of 
the hands and feet, and that these changes diminished to- 
ward the spinal cord. The gray substance of the spinal cord 
was normal ; there was a slight increase of the glia in the 
column of Goll, but not a true sclerosis, and the nerve- 
fibres were not diminished. The changes in the muscles 
consisted of simple atroph}/ of the fibres, of a loss of trans- 
verse striation, and of a proliferation of the nuclei. There 
were also some degenerated fibres and some in a condition 
of hypertrophy. It will be seen from this post-mortem ac- 
count that the changes in the spinal cord were evidently 
secondary to those in the peripheral nerves ; furthermore, 
that the changes in the muscles resembled more closely 
those occurring in the primary dystrophies than in dis- 
eases due to spinal processes. This form evidently holds 
a median position between the true spinal amyotrophies 
and the primary myopathies. For the present it is well 
to accept, with some reserve, the neural origin of the 
disease. Later investigations may lend some coloring to 
the proposition of Bernhardt to speak of the cases as spinal 
neuritic atrophies, since the ganglion cells of the anterior 
horns, the anterior nerve-roots, and the peripheral nerves, 
after all, constitute a physiological unit. Further post- 
mortem examinations of the subjects of this disease ma}' ex- 
hibit more considerable changes in the spinal cord than 
those which were found in Dubreuilh's case. I cannot con- 
sider the question absolutely settled, as it never can be bv 
a single autopsy, and would urge a careful and detailed ex- 
amination of every such case if opportunity presents itself. 



420 THE NERVOUS DISEASES OF CHILDREN. 

The general treatment of these cases will be referred to 
in connection with the discussion of the other forms of mus- 
cular disturbance. I only wish to insist once more on 
the fact that the orthopedic surgeon may in these cases pro- 
cure moderate relief, at least for a considerable period of 
time, by such measures as were employed by Dr. Gibney 
in the cases of the two boys under my immediate care. 

PRIMARY MYOPATHIES (PRIMARY MUSCULAR DYSTROPHIES). 

Our knowledge of these dystrophies is a recent acquisi- 
tion for which we are specially indebted to the brilliant 
clinical and pathological studies of Erb. The German 
neurologist not only described a new form of progressive 
myopathy, but demonstrated very clearly the relations of 
this form to the older and well-known form of muscular 
pseudo-hypertrophy. In addition to this, Erb has sub- 
jected the innumerable types of muscular atrophy to a 
healthful criticism, and has thus helped to correct many er- 
roneous views which were advanced by others. Excellent 
work has also been done by Charcot, Landouzy, Dejerine, 
Schultze, Strumpell, and Hammond. 

For a long time the Aran-Duchenne type of progressive muscular atrophy 
and muscular pseudo-hypertrophy were the only well-known forms of pro- 
gressive muscular disease. With the evidence which proved that pseudo-hy- 
pertrophy was never of spinal origin, a wide distinction was created between 
the spinal forms and the primary myopathies. As new types of muscular 
diseases were described by individual authors the question arose in each in- 
stance, whether the cases reported inclined rather to the spinal than to the 
pseudo-hypertrophic form. It is only very recently that sufficient evidence 
has been brought forth to show that all the primary myopathies are closely 
related to one another, and that the types that have been so carefully de- 
scribed in former years, are practically nothing but peculiarities in the topo- 
graphical distribution of diseases which should be included under the broad 
term of progressive muscular dystrophies. Much was at one time made of 
the occurrence of atrophy or hypertrophy, but less importance is attached to 
this point now, for we know that the hypertrophic stage represents in most 
instances an earlier stage of the disease, and that atrophy rapidly supervenes 
upon this apparent or real hypertrophy of muscular tissue. Hypertrophy 
never occurs in the spinal forms of muscular wasting * and in this respect it 

* Single hypertrophic d fibres have been found in sections of muscles from cases of 
progressive amyotrophy. 



PROGRESSIVE MUSCULAR ATROPHIES. 



421 



is a most significant symptom, but its occurrence is not a sufficient basis for 
a classification of the various types of primary myopathy. 

Before entering upon a detailed account of the primary 
myopathies, it will be well to state the chief features of 
the various types. By placing the symptoms in parallel 
columns the entire subject can be understood more readily: 

Types of Primary Dystrophies. 



Part first affect- 
ed. 

Distribution of 
hypertrophy. 

D i s t r i b u tion 
of atrophy. 



Parts remain- 
ing normal. 



Muscular Pseudo-hy- 
pertrophy. 



Legs (calves). 



Calves, rarely thighs. 



Thighs, deep muscles 
of back, shoulder.and 
scapular muscles. 
Calves during later 
period ; at that time 
also general atrophy. 

Face, forearm, and 
hand, except in last 
stages. 



Juvenile form of Pro- 
gressive MUSCULAR 

ATROPHY (ERB'S TYPE). 



Shoulder girdle. 



Muscles around shoul- 
der-girdle and pelvic 
girdle. 

Thighs, deep muscles 
of back, upper arm. 
Hypertrophied parts 
may become atrophic 
in later stage. 



Type Landguzy- 
Dejerine. 



Face and shoulder- 
girdle. 

None. 



Face muscles, includ- 
ing lips and orbicu- 
lar i s palpebrarum ; 
shoulder and scapu- 
lar muscles. 



Face, forearm, hand Forearm, hand and 
and leg muscles, ex- legs, and deep mus- 
cept in last stages. j cles of back. 



If we add to the above table the facts that in all these 
three forms there is a distinct history of heredity, or at least 
of the occurrence in various members of the same family ; 
that there are often slight and sometimes very marked quan- 
titative changes in the electrical reactions, but that there is 
rarely if ever a complete reaction of degeneration ; if we 
note, furthermore, that the reflexes may remain preserved 
for a considerable period of time and then disappear, in 
keeping with the progress of the muscular wasting, we 
shall see that there is practically no other distinction be- 
tween these various types of myopathies than the mere 
distribution of atrophy or hypertrophy. The hereditary 
type of progressive muscular atrophy as it was described 
by Leyden, is not included, simply because heredity is not 
a sufficient basis of classification, and many of his cases 
would belong more properly either under the heading of 
Erb's type, or under that of the peroneal form of progres- 



422 



THE NERVOUS DISEASES OF CHILDREN. 



sive muscular atrophy. There appears at first sight to be 
a broad gap between the cases of muscular pseudo-hyper- 
trophy and the cases of Erb's type, but these different types 
may be represented in various members of a single family. 
The relation of these types to one another is convincingly 
demonstrated by the cases of three brothers to be referred 

to in this chapter. (Figs. 106- 
108.) 

Landouzy - Dejerine have 
insisted on the right of their 
type to special consideration, 
claiming that ordinary muscu- 
lar pseudo-hypertrophy, and 
even Erb's type of disease, 
were never associated with 
an involvement of the face, 
yet it is very certain that their 
type is practically nothing 
more than that described by 
Erb, plus involvement of the 
face muscles. Landouzy-De- 
jerine also denied that an at- 
rophy of the face muscles 
was ever associated with typ- 
ical pseudo-hypertrophy ; but 
Westphal first published a 
case which showed that the 
face muscles are occasionally 
affected in cases of typical 
pseudo -hypertrophy, and I 
was able, some years ago, to 
record a case which was a 
typical representative of mus- 
cular pseudo- hypertrophy, 
which passed through the 
stage defined by Erb's type, 
and in which the muscles of 
the face were also involved. There is, therefore, no suffi- 
cient reason to retain the Landouzy-Dejerine type as a sep- 
arate -form of disease. 




Fig. 106. — Oldest Brother, aged Sixteen 
Years, exhibiting Atrophy following 
Pseudo-Hypertrophy of the Calves, 
and Extreme Atrophy of Shoulder 
Muscles. Boy unable to move from 
chair or to hold himself erect (late 
stage of ' ' pseudo-hypertrophy" ) . Figs. 
106-108 represent three brothers. 



PROGRESSIVE MUSCULAR ATROPHIES. 



423 



The characteristic symptoms of the various types may 
now be described. 

Muscular Pseudo-hypertrophy. — As described many 
years ago, above all by Meryon, Duchenne, and Gowers, 
pseudo-muscular hypertrophy is characterized by its oc- 
currence in early youth. 
Boys are affected some- 
what more frequently 
than girls ; but although 
affecting boys, the dis- 
ease is inherited almost 
invariably through the 
mother. The first symp- 
toms are, a weakness in 
the muscles of the leg 
and an early increase in 
the size of the calf mus- 
cles. In rare instances 
the hypertrophy may be- 
gin in the thigh muscles. 
The gait is waddling, and 
the child soon finds diffi- 
culty in walking up and 
down stairs, in climbing 
on chairs, in rising from 
the floor or from any re- 
cumbent posture. In the 
earlier stages of the dis- 
ease the patient rises from 
the floor by dint of great 
effort (see Fig. 109) and 
by " climbing up upon 
himself." In later stages 
of the disease the patient, 
if put on the floor, lies 
absolutely prostrate and 
is not even able to raise 
the head from the floor. 

Sitting up without support may be entirely impossible. As 
the weakness and atrophy increase, the patient becomes more 




Fig. 107. — Second Brother, aged Thirteen and 
a Half Years, exhibiting Hypertrophy of 
Calves, of Gluteal Muscles, and of Muscles 
about the Shoulder Girdle ; Distinct Atrophy 
of Arm Muscles (Erb's Type, or Juvenile 
Form of Progressive Muscular Atrophy). 
All the muscles are now beginning to atro- 
phy ; boy is only a little less helpless than his 
older brother. 



424 



THE NERVOUS DISEASES OF CHILDREN. 



and more helpless, is unable to stand or to walk, becomes 
either bedridden, or is compelled to sit in a chair and even 
loses the use of the upper extremities ; is not able to raise 




Fig. 108.— Youngest of the Three Brothers, in the Earlier Stage of Pseudo-Hypertro- 
phy. (For the photograph of this boy I am indebted to the courtesy of Dr. Collins.) 



the arms, and may have no use of anv of the muscles, except 
the small muscles of the hand. In the two boys (Figs. 106, 
107) under my observation, a climbing up of the hand along 



PROGRESSIVE MUSCULAR ATROPHIES. 



425 



the head in order to get the arm into the erect position was 
a very characteristic feature. 

In addition to the hypertrophy of the calf muscles, we 
now know that there is apt to be atrophy of the muscles of 
the thigh, of the arm, and the shoulders ; the scapular mus- 




FiG. 109.— Boy with Pseudo-Hypertrophy attempting to straighten Himself. Same 

patient as in Fig. 108. 



cles, and at a very early stage of the disease the serrati, the 
latissimus dorsi, and the pectoralis major, are often wasted. 
The forearm muscles and the hand muscles are rarely af- 
fected. The disease is often associated, as the other forms 
of myopathy may be, with symptoms of a general degener- 
ation ; thus I have found nystagmus, lisping speech, and a 
moderate degree of imbecility in not a few of these cases. 



426 



THE NERVOUS DISEASES OF CHILDREN 



Erb's Type, or the Juvenile Form of Progressive 
Muscular Atrophy. — According- to Erb's own summary, 
this type is characterized by progressive wasting with 
weakness of certain groups of muscles, beginning either in 
childhood or early youth, involving, as a rule, the muscles 
of the shoulder girdle, the upper arm, the pelvic girdle, the 
thigh, and the back ; the forearm and leg muscles remain- 
ing intact for a very long time. The atrophy may be asso- 
ciated with true or pseudo-hypertrophy of some muscles. 

The pectorals, trapezii, la- 
tissimi dorsi, the serrati, the 
rhomboids, as well as most 
of the upper-arm muscles 
and supinators are apt to 
be wasted ; while the del- 
toids, supraspinal, and in- 
fraspinati are either normal 
for a long time or hyper- 
trophied. There are no 
fibrillary contractions and 
no reaction of degenera- 
tion ; no sensory or visce- 
ral disturbances. (See Fig. 
107.) 

A few years ago, I was 
inclined to consider Erb's 
type a great rarity in this 
country ; it is unquestion- 
ably the least frequent of 
all the forms of progressive muscular atrophy which we 
have occasion to see in clinics or private practice ; but I 
have seen at least a dozen cases of this form within the 
last five years, from which the reader may gather the fre- 
quency or infrequency of the disease; but there is no doubt 
that many cases exist which have not been reported, simply 
because they have not been properly recognized. 

The Facio-Scapulo-Humeral, or Laxdouzy-Deje- 
rixe Type. — This type includes cases in which the atrophy 
begins early in life, and, as a rule, in the muscles of the face, 
giving rise to what the authors have termed the "fades my- 




Fig. iio. — Patient with Landouzy-Deje- 
rine Type. Indication of bouche de ta- 
pir ; patient cannot show upper teeth, nor 
close eyes. (See also Fig. 8.) 



PROGRESSIVE MUSCULAR ATROPHIES. 427 

opatJiiqtie ;" the lips are considerably thickened and consti- 
tute the boucJic dc tapir, or tapir mouth. Later on in the 
course of the disease the atrophy spreads to the shoulder 
and arm muscles. The supraspinati and infraspinati, the 
subscapulars, and flexors of the hands and lingers remain 
normal. Among these muscles that remain normal it may 
at once be noted are several which are distinctly hypertro- 
phied in Erb's type. In the Landouzy-Dejerine type the 
muscles of deglutition, of mastication, the respiratory and 
laryngeal muscles, as well as the ocular muscles, remain nor- 
mal. In exceptional cases the disease may begin in the 
shoulder or arm muscles, or even in the lower extremities, 
The disease is distinctly hereditary. Fibrillary contrac- 
tions and reaction of degeneration are never present. 

I have given these symptoms as nearly as possible as 
they were stated by the authors themselves in order to do 
full justice to their cause, but it will be evident, without 
further argument, that, with the exception of the involve- 
ment of the face, there is very little distinction between 
these cases and those of the juvenile form of progressive 
muscular atrophy. 

The various types of progressive myopathy are suffi- 
ciently illustrated by Figs. 106-1 10, which supply additional 
evidence in favor of the intimate kinship existing between 
these various types. 

Figs. 106 and 107 represent two brothers who have been under my obser- 
vation for many years ; the one is an example of muscular pseudo-hyper- 
trophy with atrophy of shoulder and trunk muscles ; the other boy's disease 
began as a muscular pseudo-hypertrophy, but the hypertrophy of the muscles 
above the shoulder and pelvic girdles constitute it a most pronounced case of 
Erb's type — the juvenile form of progressive muscular atrophy. A third 
brother is now in the state of pseudo-hypertrophy (Fig. 108). After a lapse 
of years the pseudo-hypertrophy may disappear, and all three brothers will 
then bear the closest resemblance to each other. Fig. no is a photo- 
graph of the patient afflicted with the Landouzy-Dejerine type of muscular 
atrophy. 

To these I wish to add a brief history (published in iSqo\ of a case 
which was one of unusual importance, as the patient presented the combined 
symptoms of all known types of primary myopathy. A young man. twenty 
years of age, whose history is entirely negative, has noted, since early childhood, 
a peculiarity about his face ; he was not able to whistle as well as other boys. 



428 THE NERVOUS DISEASES OF CHILDREN. 

and as long as he can remember his face appeared twisted to one side, at 
least while speaking. It was not until one year before my examination that 
he became aware of any further trouble. He claims to have struck his 
shoulder, and since that time to have noticed a weakness of both upper ex- 
tremities. He was employed by a surgical-instrument maker and had to lift 
heavy boxes, but this he is no longer able to do. 

During the examination, was asked to whistle ; he could not do it, nor 
could he keep his eyelids tightly pinched. The condition of his muscles may 
be summarized as follows : 

Wasted : Both pectorals, major and minor. Both serrati, right more 
than left. Both latissimi dorsi, left more than right. Levatores anguli 
scapulae, right more than left. Rhomboids, left more than right. Both tra- 
pezii (middle and lower third), left more than right. Both biceps muscles 
and both brachiales-antici. Both triceps muscles, right more than left ; the 
supinator longus of both arms, right more than left. Anterior thigh and leg 
muscles, left more than right. Posterior thigh muscles (thin). Orbicularis 
palpebrarum of each side and the orbicularis oris. 

Normal : Back muscles, forearm and hand muscles, gluteal muscles, and 
muscles of foot. 

Hypertrophied : Deltoids, infraspinati, supraspinati, and calf muscles, but 
the right calf is beginning to waste. 

There were no fibrillary contractions in any of the affected muscles. All 
the muscles, including those wasted and those hypertrophied, responded to 
both currents in proportion to the quantity of normal contractile fibre that 
each muscle retained. There was no reaction of degeneration in any muscle. 
The knee-jerks were present. There was no ataxia, no disturbance of sen- 
sation, and there was not a single symptom pointing to an involvement of the 
central nervous system. 

From the condition of the calves, there might be some reason to class this 
case among the pseudo-hypertrophies ; from the appearance of the shoulder- 
girdle and the thinness of the upper arm, we might rank it with Erb's juve- 
nile form ; and if we take the face into consideration, we might classify it 
with the Landouzy-Dejerine type of progressive muscular atrophy. It does 
not quite tally with the older accounts of pseudo-hypertrophy ; for although 
the shoulder muscles are sometimes involved in such cases, it is exceptional 
to have both shoulder and face muscles affected. From Erb's form it is dis- 
tinguished by the involvement of the face muscles, and from the ordinary 
cases of the Landouzy-Dejerine type this case is to be distinguished by the 
additional involvement of the calves. It will not do to suppose that the boy 
is affected with three different diseases ; it is much more to the point to state 
that the symptoms in this case prove that the three distinct forms practically 
represent subdivisions of one and the same disease, and that the primary 
muscular disease was so fully developed in this patient that he practically 
represented all the known types of progressive myopathy. We see by this 
case, too, how wrong it is to make too much ado over the varying distribu- 
tion of atrophies or hypertrophies. 



PROGRESSIVE MUSCULAR ATROPHIES. 429 

Diagnosis. — It is much more difficult to differentiate 
between the various forms of progressive muscular dys- 
trophies than to distinguish between them and other dis- 
eases. The factor of heredity, the occurrence in families, 
the absence of fibrillary twitchings and of changes in elec- 
trical reaction, and above all the slowly progressive char- 
acter of these diseases and their onset in early life, will 
scarcely permit of any confusion with other diseases. If 
the patients are examined later in life, after extensive atro- 
phy has supervened upon preceding hypertrophy, if the 
atrophic paralysis is so extreme that the patient is bedrid- 
den and that there are practically no muscles which re- 
spond to the electrical current, if all the reflexes are absent, 
there may be considerable difficulty in differentiating be- 
tween such a condition and that of chronic poliomyelitis. 
But even at such an advanced period of the disease the 
former history of the patient will help to establish a correct 
diagnosis. 



Mention should, however, be made of one other condition which I have 
met with but twice, in which the question has come up whether the patient 
was suffering from a form of primary muscular dystrophy. Both of these 
cases were instances of what I would wish to call physiological hypertrophy. 
The one was the case of a physician who had attained unusual muscular 
development in his efforts to correct phthisical tendencies, and the second 
was the case of a brother of a well-known physician who, through inordi- 
nate exercising at lawn tennis had produced a hypertrophy of the shoulder, 
arm, and forearm muscles of the right side. In both these cases I was con- 
sulted because of a weakness which had followed upon this unusual hyper- 
trophy. In the case of the physician first referred to, several muscles (among 
them the infra and supra spinati, and the deltoid) had begun to atrophy dis- 
tinctly. The possibility of the development of some form of progressive 
muscular atrophy was entertained by others in both cases, but I was certain 
that this grave prognosis was not justified, for the entire development of the 
trouble, the onset late in life, and the occurrence of these conditions in per- 
sons with a clear family record furnished the best evidence that the condition 
of these two patients was the result of over-exercise and nothing more. 

The point of greatest interest in both these cases is the surprising weak- 
ness of the muscles in spite of the hypertrophied condition. It would seem 
that any muscular fibre forced to an unnatural growth (hypertrophy) is 
likely to succumb in the struggle. In both these cases I insisted on absolute 
rest of the affected parts and on the use of common sense in physical exer- 
cise. In the case of the physician all the muscles have returned to a norma! 



43° THE NERVOUS DISEASES OF CHILDREN. 

state and have remained so for fully three years ; in the second case the 
normal strength of the arm has returned, and there is no sign of an impend- 
ing atrophy. 

The points of differential diagnosis between the spinal 
forms of progressive muscular atrophy and the primary 
dystrophies have been insisted upon over and over again. 
The rule is that in spinal cases the affection is not hered- 
itary and generally begins in the upper extremity ; there 
are fibrillary contractions, and marked changes in electri- 
cal reaction ; while in the dystrophies heredity is the most 
prominent factor, the diseases begin early in life, there are 
no fibrillary contractions, and the electrical reactions re- 
main normal or nearly so throughout the entire course of 
the disease until the stage of extreme atrophy is reached. 

After the consideration of these cardinal symptoms there should be no 
real difficulty in distinguishing between these two principal forms of progres- 
sive muscular wasting ; yet cases appear every now and then in which the 
symptoms are so distributed that it is impossible to classify them by adhering 
to these cardinal symptoms. The most interesting case in point is unquestion- 
ably the one reported by Striimpell. In this patient the disease did not begin 
until the age of twenty-nine, in the fingers of the right hand, but there was a 
strong history of heredity, his mother having suffered from a similar disease. 
The atrophy spread from the small muscles of the right hand to the muscles 
of the shoulder, and later on to the muscles of the opposite arm. The deep 
muscles of the spine, the glutei, and the thigh muscles remained entirely in- 
tact, after a period of more than eleven years. The symptoms just stated 
pointed to a spinal form, rather than to a pure myopathy ; the heredity 
was, however, more in keeping with the primary dystrophies, and the entire 
absence of fibrillary movements as well as of changes in the electrical re- 
actions would have inclined one to place the case in the category of primary 
myopathies. The histological character of the muscular tissue was also more 
like that described in cases of primary myopathies, yet at the autopsy de- 
cided changes were found in the spinal cord, in the anterior nerve-roots, and 
in a number of the peripheral nerves. Thus the impropriety of classing such 
a case exclusively under one heading or the other was clearly demonstrated, 
and I think Striimpell quite right in insisting that the chief value of his case 
was in showing that these cases of progressive muscular atrophy, whatever 
form they take, belong to the order of hereditary systemic diseases, and that 
it is largely a matter of chance or else due to causes still unknown whether 
the peripheral or the more central portion of the second division of the mo- 
tor tract becomes the chief seat of the disease. A case of Savill's shows 
that the symptoms of an amyotrophy may coexist with those of a myopathy 
of the Landouzy-Dejerine type. 



PROGRESSIVE MUSCULAR ATROPHIES. 43 1 

Pathology. — The designations, " primary myopathy," 
or " primary muscular dystrophies," which have now been 
universally accepted for the diseases under consideration, are 
meant to imply that the origin of the disease is in the mus- 
cular system itself, and that it is not due to changes either 
in the peripheral nerves or in the spinal cord. With regard 
to the more recently described forms of primary muscular 
dystrophy, there has never been any doubt as to the non- 
spinal origin, but a long and hot discussion was waged over 
the spinal origin of muscular pseudo-hypertrophy. In 1888 I 
analyzed all the cases of muscular pseudo-hypertrophy that 
were accessible at the time, and found that of twenty-five 
cases which had been reported, eight had to be excluded 
because the spinal cord was not examined microscopically, 
or because the examination was not properly made. Of 
the seventeen remaining cases, the spinal cord and anterior 
nerve-roots were found absolutely normal in twelve, and in 
five others the changes that were found could not be held 
responsible for the changes in the muscles. A similar con- 
clusion has been reached by other authors, and at the pres- 
ent time no author of repute has ventured to fall back 
upon the older theory of the spinal origin of muscular 
pseudo-hypertrophy. Such slight changes as were observed 
by some — the diminution, for instance, in the number of 
ganglion cells or slight changes in the contours of these 
cells — can now be sufficiently explained on the supposition 
that all such changes are secondary to the peripheral mus- 
cular trouble. 

The histological condition of the atrophied or hypertro- 
phied muscles has been studied with particular care, for it 
was supposed for a very long time that the diagnosis of a myo- 
pathy or of an amyotrophy could be based safely enough 
upon histological appearances ; but we shall see that this 
hope of finding some absolute point of differentiation be- 
tween these two conditions can no longer be entertained. 
In muscles atrophied from spinal lesions, the following were 
supposed to be the chief changes: a loss of striation of the 
muscular fibres and a narrowing of the same, an increase 
in the number of muscle nuclei, and possibly segmentation 
of the nuclei, granular or fatty degeneration of the fibres. 



432 THE NERVOUS DISEASES OF CHILDREN. 

and occasionally globules of fat between the muscle fibrils. 
Some or all of these changes are present according to the 
length of time that the atrophy has existed, but all of these 
changes have also been found in spinal forms, as well as 
in primary dystrophies. The increase in the nuclei is not 
so great in the dystrophies as in the amyotrophies. In the 
spinal forms it was thought that hypertrophied fibres were 
never found, while they are extremely common in the 
purely muscular types. But Mueller and others have re- 
corded exceptions to this rule and have proved the presence 
of hypertrophied fibres even in cases of poliomyelitis of 
old standing. A large number of hypertrophied fibres in 
a given section of a muscle is, nevertheless, more frequent 
in the primary muscular dystrophies than in the spinal 
atrophies. 

The next question that arose was whether it was possible by histological 
examination to differentiate between the various primary myopathies. In 
muscular pseudo-hypertrophy we have, as a rule, a narrowing of the fibres 
with changes in their contour, granular or fatty degeneration of the fibrils, 
and accumulation of fat globules between them, and increase, without marked 
proliferation, of the connective tissue. Hypertrophied fibres can be found 
scattered in between fibres of normal dimensions, or between those that have 
evidently undergone atrophy. A slight increase in the muscle nuclei is often 
found, but unusual increase is suspicious of a spinal origin. Jacoby thought 
that the disease consisted in the main of a chronic inflammation invading 
both the perimysium and the muscle tissue, and was inclined to term the 
process a myositis progressiva hyperplastica, but his views and his histo- 
logical findings have not been corroborated by others. Westphal, in a very 
typical case of pseudo-hypertrophy, found, on post-mortem examination, 
enormous increase of adipose tissue in which the muscle fibrils were nearly 
of normal size, increase of the interstitial connective tissue, no hyper- 
trophied fibres, and strands of connective tissue occasionally passing 
through the fatty parts ; a few of the groups of muscle fibres appearing 
to be strangulated by the strands of connective tissue. In sections from 
a case which I have recently had occasion to examine, the fibres were found 
to be of varying size and there was a distinct proliferation of the muscle 
nuclei. Schultze, in a case which stands midway between pseudo-hyper- 
trophy and Erb's juvenile form, found, in addition to peculiar giant-cell for- 
mations, a large number of fat-cells in the muscular tissue, an increase of the 
connective tissue, and remnants of hypertrophied, normal, and atrophic fibres, 
and an enormous increase of nuclei, which the author thought greater than 
in the ordinary cases of pseudo-hypertrophy. He also described the occur- 
rence of vacuoles which were in all probability not due to the hardening 



PROGRESSIVE MUSCULAR ATROPHIES. 



433 




Fig. hi. — Changes in Muscular Tissue in a Case 
of Primary Dystrophy. (Erb. ) a, Above altered 
blood-vessel ; for other lettering see text. 



process. Hitzig's observations were of special interest for a time, as he 
examined four cases most carefully. He concluded that " the primary and 
most important change in juvenile atrophy is not an interstitial process, but 
decidedly parenchymatous, and according to the intensity of the disease is 
represented by slight or ex- 
cessive hypertrophy of the 
fibres . . . The anatom- 
ical changes in pseudo-hyper- 
trophy, on the other hand, 
are characterized by active 
changes in the connective 
tissue." This distinction be- 
tween the histological changes 
in pseudo - hypertrophy and 
Erb's form of primary myop- 
athy has not been borne out 
by others, and if I am not 
mistaken, Hitzig has with- 
drawn his former views. The matter was definitely settled by more recent 
and extremely thorough studies of Erb (see Figs, in and 112). He proved 
that in all cases of progressive muscular dystrophies, whether of one type or 
another, the changes in the muscular tissue were very similar, and that 
such changes as did occur were simply due to different stages of the dis- 
ease. Erb showed (Fig. in) that hypertrophy and atrophy of fibres, marked 
proliferation of the nuclei, vacuolization (near b), and segmentation (c) of 
fibres were the chief changes, and that these occurred in all possible forms of 
primary dystrophies, and of these phenomena the hypertrophy of the fibres 

seemed to be the first to ap- 



pear. 

From a case of the Lan- 
douzy - Dejerine type (Fig. 
no), I excised a large piece 
of the infra-spinatus muscle, 
and in doing this a piece of 
the nerve as it enters the 
muscle was accidentally re- 
moved. On histological ex- 
amination of the specimen, 
prepared for me by Dr. 
Wiener, we found a consid- 
erable amount of fatty tissue between and around the muscular fibres (Figs. 
113, 114) much atrophy, but no hypertrophy of the muscle fibres ; there was 
also distinct evidence of a marked degeneration of the nerves. The finding 
was a very singular one, but it will not do to draw too broad an inference 
from a single case. At all events, the participation of the nerve (whether in 
primary or secondary fashion) in a disease supposed to be a purely muscular 
affection is worthy of note. 
2S 




Fig. 112. — a, Normal Fibres; b, Hypertrophied 
Fibres ; c, Atrophied Fibres and Fat. (Erb. ) 



434 THE NERVOUS DISEASES OF CHILDREN. 

After a very long discussion on the histological changes 
in progressive muscular dystrophies, Erb concludes that 



:\ 



■fZ* 



<5# 






Fig. 113. — Section of Infra-Spinatus Muscle from a Case of Landouzy-Dejerine Type, 
showing Deposits of Fat and Degenerated Bundles of Nerves. (Low power. Draw- 
ing made after specimen stained according to Van Gieson.) 

these dystrophies are forms of a tropho- neurosis, which 
may occasionally be the result of functional disturbance of 
trophic centres ; and that such disturbance may be either 



Fig. 114. — A Part of the Nerve Bundles more highly magnified, showing Degenera- 
tion of Nerve Fibres. (See preceding figure.) 

primary or secondary to spinal lesions — a conclusion not 
unlike that reached by Striimpell a few years later in the 
article referred to above. 



PROGRESSIVE MUSCULAR ATROPHIES. 



435 



Duration. — All these forms of muscular dystrophias take 
a relatively slow course, but the period of time that elapses 
before the stage of utter helplessness is reached varies very 
much in different individuals. It is safe to say that those 
suffering from muscular pseudo-hypertrophy are the ones 
most apt to be completely crippled at an early day. Two 
or three years often suffice to make the patient entirely help- 
less. Erb's juvenile form 
and the Landouzy-Dejerine 
type progress more slowly 
— a number of years elaps- 
ing before the legs become 
involved. I have seen one 
case of Erb's form which 
has lasted over thirty years, 
and another of the typical 
muscular pseudo-hypertro- 
phy, which has lasted at 
least fifteen ; but if death 
does not ensue from the 
disease itself, patients may 
be carried off by any slight 
intercurrent disease. 

Treatment. — Interest- 
ing as these diseases are 
from a medical and scien- 
tific point of view, they are 
unfortunately most unsat- 
isfactory as regards the re- 
sults of treatment ; and yet 
there is no need of despairing and declaring that nothing 
can be done in the earlier stages of these troubles. Con- 
tractures of muscles and deformities of various parts are 
extremely annoying and prevent the patient from getting 
about freely, or without the use of crutches or wheeling- 
chairs. I wish to plead for the early and active interfer- 
ence of the orthopedic surgeon. As the diseases are at 
times but very slowly progressive, the correction of de- 
formities by surgical measures may bring relict for a con- 
siderable period of time. Excellent though temporary 




Fig. 115.— Boy with Defective Development 
of Scapula and Shoulder Muscles. 



43<5 



THE NERVOUS DISEASES OF CHILDREN. 



results were obtained in several patients whom I have 
treated in conjunction with Dr. Gibney. His results in the 
cases of the peroneal form of progressive muscular atrophy 
make one a little hopeful that still better results may be at- 
tained in the future in this and other forms of muscular 
atrophy by similar or even bolder measures. I am confident 
too that improvement can be secured by complete rest 

and by insisting upon the 
greatest care in the exer- 
cise of atrophied or hyper- 
trophied muscles ; also by 
the diligent and prudent 
use of massage and elec- 
tricity in these cases. The 
latter agent has a whole- 
some influence upon the 
condition of muscles whose 
nutrition has been altered, 
and is furthermore a con- 
venient form of moderate 
exercise. In the case of a 
boy suffering from the 
Landouzy - Dejerine type, 
all the symptoms were 
brought to a standstill, and 
a decided improvement 
has been effected by the 
careful administration of 
electricity. If the stage of 
atrophy and helplessness 
has been reached, nothing more can be done than to make 
the patient as comfortable as possible. 




Fig. 116. 



TOTAL ABSENCE AND EARLY ATROPHY OF MUSCLES. 

This condition is suggested by the preceding discussion 
of the primary muscular dystrophies, and may as well be 
referred to in this connection as in any other. The condi- 
tion is a rare one. The following case, which I have seen, 
suggests a similar condition, and at the same time shows 



PROGRESSIVE MUSCULAR ATROPHIES. 437 

that the defect may include bony parts as well as the mus- 
cles covering them.* 

The boy whose condition is represented in Figs. 115 and 
116 was seen by me a few years ago. He was then six 
years of age. The mother stated that she had noticed dur- 
ing the past year that the right shoulder was higher than 
the left. On examination we found that there was shorten- 
ing of the right scapula, the longest diameter being 4 ctm. 
less than that of the left, and there was insufficient develop- 
ment of the right supra- and infra-spinati of the latissimus 
dorsi, and of the pectoralis major. It will be noted that 
these are the very muscles which are often involved in 
cases of primary muscular dystrophies. The condition is ol 
some interest as showing that the muscles which are most 
subject to disease are also found congenitally defective. 
Whether this defect of development is due to some periph- 
eral nerve anomaly, or whether it is the result of defec- 
tive development of the spinal-cord cells, cannot be stated. 



BIBLIOGRAPHY. 

PROGRESSIVE MUSCULAR ATROPHIES. 

Bernhardt : Virchow's Arch., vol. 133, p. 259. (Contains literature.) 

Blocq et Marinesco : Type Landouzy-Dejerine. — Case with Autopsy. Arch, 
d. Neurol., 1893. 

Buss : Berl. Kl. Wochenschr. , 1887, No. 4. (Progressive dystrophies.) 

Charcot-Marie : Rev. de Medecine, 1886. (Peroneal form.) 

Dejerine et Sottas : Progres. Med., 1893, No. 12. Hypertrophic and Progres- 
sive Neuritis in Childhood. 

Dreschfeld : Rare Forms of Muse. Atrophies. Brain, July, 1885 and 1S86. 

Dubreuilh : Revue de Medecine, 1890, p. 441. 

Erb : Dystrophia Muscularis Progressiva. Volkmann's Samml., 1S90, Nos. 
1 and 2 ; Deutsch. Zeitschr. f. Nervenhk., Bd. I., 1891 ; Neurolog. Cen- 
tralblatt, 1889. 

Higier : Deutsch. Med. Wochenschr., September 14, 1S93. (Good summary.) 

Hoffman : Ueber chr. Spinale Muskelatrophie im Kindesalter auf familiarer 
Basis. Deutsche Ztschr. f. Nervenheilkunde, Bd. III., p. 427. 

Heubner : Wagner Festsschrift, Leipzig, 1887, 

Landouzy-Dejerine : Revue de Medecine, vol. v., pages 81 and 251. 

Osier : Archives of Medicine, New York, 1880. 

* The literature of this special subject is given in part in Erb's article (Neurolog. 
Centralbl., 1889), describing a case in which both trapezii were wanting. 



438 THE NERVOUS DISEASES OF CHILDREN. 

Raymond : Atrophies Musculaires, etc. Monograph, Paris, 1889. 

Reinhold : Zeitschrift f. Nervenheilkunde, Bd. IV. 

Sachs, B. : Brain, January, 1890, Peroneal Form or Leg Type; New York 
Medical Journal, December, 1888 ; Philadelphia Medical News, June 21, 
1890. 

Sacki : Progr. Neurotische Muskelatrophie, Berl. Kl. Wochenschr., July 24, 
1893. 

Savill (London) : Nouv. Iconographie de la Salpetriere, No. 3, 1894. 

Schultz: Monograph, 1886. 

Stewart : in Keating's Cyclopaedia. " Primary Muscular Atrophies." 

Struempell : Zeitschr. f. Nervenheilkunde, Bd. III. 

Stude : Zur Different. Diagn. d. progressive Neurotischen Muskelatr., Berlin, 
1892. 

Thomson & Bruce : Edinburgh Hospital Reports, vol. i., 1893. Prog. Muse. 
Atrophy in a Child with a Spinal Lesion. 

Tooth : Brain, vol. x. (Peroneal form.) 

Werdnig : (Early infantile hereditary form.) Arch. f. Psych. Bd. XXII., 2. 

Westphal : Charite Annalen, 1885. 

Zimmerlin : Zeitsch. f. Kl. Med. Bd. VII., 1884. (On Hereditary Progres- 
sive Muscular Atrophy.) 



CHAPTER XXIV. 

MALFORMATIONS AND CONDITIONS DUE TO DEFECTIVE 
DEVELOPMENT OF THE CORD. 

Arrest of, or disturbance in, the development of the 
spinal cord is generally associated with similar conditions 
affecting the brain. These spinal-cord conditions have, with 
the exception of spina bifida, very little clinical interest. 
The chief malformations are the following : 

Amyelia, or Entire Absence of the Spinal Cord, is associated 
with a condition of anencephaly, and the absence of both may be designated 
as amelyencephaly. In some instances there is tolerably normal develop- 
ment of the cord, however, while every trace of the brain may be wanting. This 
defect of the spinal cord is in some instances a simple form of agenesis, in 
other cases a start has been made in the development of the central nervous 
system, but an unusual accumulation of cerebro-spinal fluid at a later period 
of foetal life has destroyed the neural axis. In forms of amyelia there is at 
times also a defective development of the vertebras. If the defect is due to a 
spinal dropsy we are able to. trace, after the birth of the child, the vertebral 
canal and the spinal membranes which are distended by the excessive ac- 
cumulation of fluid. 

Atelomyelia is the term by which the older authors designated a par- 
tial lack of development, such as the entire defect of the pyramidal tracts, or 
a slight defect in the substance of the cord which occurs at the site of the 
spina-bifida. A similar defect is at times observed in the lower portions of 
the spinal cord, which is generally associated with some congenital defect in 
the development of the lower extremities. The only form of atelomyelia 
which is of any practical significance, is the one referring to the defective de- 
velopment of the pyramidal tracts, and that has been considered in the chap- 
ter on hereditary spastic paralysis. 

Asymmetry of the Cord has been described in a few instances. It is 
probably due to defective development of some of the systems of the spinal 
cord, particularly of the pyramidal tracts on one side of the cord, and not on 
both. 

Heterotopia is a condition which has aroused considerable interest of 
late. It denotes malposition of the gray matter of the cord ; parts of which 
are found scattered among the systems of white fibres, either in the lateral 



440 THE NERVOUS DISEASES OF CHILDREN. 

columns or even in the posterior columns. Van Gieson has shown that het- 
erotopia can easily be caused by careless manipulations of the spinal cord 
during post-mortem removal from the body. He throws some doubt upon 
the existence of any such condition as heterotopia during life. While it may 
be conceded that many of the cases that have been reported as heterotopia, 
were, in all probability, artificially produced, we cannot deny the occasional 
existence of such a condition : for it has been found when the spinal cord has 
been most carefully removed, and, furthermore, if it were invariably an arti- 
ficial condition, we would surely find it in many more spinal cords than is 
actually the case. 

A small cord or micromegaly has often been noted ; in cases of hereditary 
cerebellar ataxy for instance. A normal adult cord has a diameter of 6 to 9 
mm. in the dorsal vertebrae; 8 to 11 mm. in the upper cervical ; 15 mm. in 
the cervical, and 12 mm. in the lumbar enlargement. 

Diplomyelia denotes a double cord. This condition is at times found 
in monstrosities of various kinds, but occasionally in children who have ex- 
hibited no spinal symptoms during life. It is due to a defective develop- 
ment of the spinal cord, and is to be attributed chiefly to an abnormal dilata- 
tion of the central canal. In the first months of foetal life the central 
canal is relatively wide, and if normal development takes place the canal 
becomes smaller and smaller as time goes on. If this retraction does not 
take place, the substance of the cord remains divided and we practically 
have a double structure. This division is, however, never complete, except 
in a few cases associated with anencephaly. This diplomyelia need not in- 
volve the entire cord. At times it is restricted to a few segments only. A 
double central canal is not a great rarity. It denotes an arrest in the normal 
development of the cord and as a rule involves only a part of the medulla 
spinalis. The two canals lie side by side. The relation which these defects 
of the central canal bear to syringo-myelia is very evident and need not be 
dilated upon. But it will be remembered that there is some doubt whether 
syringo-myelia represents an arrest in the development of the spinal struct- 
ures, or a destruction of parts that have been normally developed. If the 
ependymal lining of the central canal is entirely normal the syringo-myelia is 
likely to be a congenital condition and not one due to disease. The disten- 
tion of the central cavity has been described as hydrorrachis interna. In 
contradistinction to this we have the condition of 



External Hydrorrachis and Spina Bifida. — This 
is by far the most important of all the congenital anomalies 
of the spinal cord, and is by no means a great rarity, oc- 
curring in about one of every thousand cases. Spina 
bifida denotes a condition in which there is an unusual ac- 
cumulation of serous fluid in the vertebral canal either 
between the pia mater and the arachnoid or between 
the arachnoid and the dura. Associated with this in- 



DEFECTIVE DEVELOPMENT OF THE CORD. 



44: 



crease of fluid is a cleft in the vertebrae due to an ab- 
sence of the vertebral arches, and in some instances the 
bodies of the vertebrae are wanting. If there is no external 
prominence the condition is called spina bifida occulta. 
Recklinghausen insists that the dura also is cleft in the ma- 
jority of cases. Through this cleft in the vertebras the 
spinal membranes or the cord may protrude, and accord- 
ing to the parts protruded we can distinguish three kinds of 
spinal hernia : First, meningocele ; second, meningo-myelo- 
cele ; third, syringo-myelocele. The first two are much 
more common than the last named. The annexed figures 
will illustrate the condition better than any verbal descrip- 



a b ca 





Gbca 




Fig. 117. 



■Meningocele, Meningomyelocele, and Syringo-Myelocele. a, vertebral 
walls ; b, cord ; c, membranes. (Dana.) 



tion would. In all of the cases the wall of the sac is lined by 
the arachnoid, but not invariably by the dura. The pia is 
not part of the sac-wall unless there is also an internal 
hydrorrhachis or hydromyelus. In some of them the spinal 
cord as well as the nerve-roots protrude into the sac. In 
others again only a few nerves are found in it, the tumor 
contains cerebro-spinal fluid, some connective tissue, and, as 
a rule, considerable fat. 

Etiology. — Spina bifida is caused by defective closure 
of the vertebral arches, formed from the mesoblast. 

This is a primary defect in development, and not caused 
by dropsy of the spinal cord, as was once supposed to be 
the case. 

Normally the vertebral column is closed from above 



442 



THE NERVOUS DISEASES OF CHILDREN. 



downward. It is natural, therefore, that the defect should 
be found most frequently in that part (the lumbar seg- 
ments) Avhich is the last to close. The external tumor is 
generally to be seen in the region of the second to fifth 
lumbar vertebrae. There is distinct fluctuation in the ma- 
jority of cases ; the size may vary from that of an egg to 

the size of a child's head. The 
skin of the tumor may be normal ; 
in some cases it is thinned out, 
particularly if the tumor grows 
rapidly. Ulcers are occasionally 
formed ; the skin may be ruptured, 
but the dura continues to act as 
the wall of the sac. In many cases 
there is a narrow opening between 
the sac and the spinal canal. At 
times there is a complete constric- 
tion at this point, giving rise to 
an ordinary cystic formation which 
may contain some of the spinal 
structures that have been separated 
from the main contents of the spi- 
nal canal. 

Symptoms. — The chief symp- 
toms are those of increased press- 
ure in the spinal and cranial cavi- 
ties, and such as point to a direct 
interference Avith the functions of 
the spinal cord. Children with 
spina bifida are often poorly devel- 
oped, and mentally deficient. If 
the child lives long enough it pre- 
sents spastic or flaccid paralysis of 
the lower extremities with anaesthesia and with or with- 
out atrophy. Talipes varus is frequent. Remak described 
a form of club-foot associated with spina bifida, differ- 
ing from congenital club-foot. The child is late in learn- 
ing to walk and has little or no control over the 
sphincters of the bladder and rectum. Ectopia of the 
bladder and other viscera, defective development of the 




Fig. 118. — Child of Seven Years, 
with Spina Bifida and Deform- 
ity of the Feet. 



DEFECTIVE DEVELOPMENT OF THE CORD. 443 

lower extremities, are frequent complications of spina 
bifida. 

Diagnosis. — The condition is easily recognized ; it is to 
be differentiated from congenital and other tumors which 
occur often enough in the lumbo-sacral region and may be 
connected either with the substance of the cord or with the 
spinal membranes. 

Fibroma, sarcoma, myxoma, hygroma, and echinococ- 
cus cysts are the forms of tumor which occur common- 
ly in this region. It is of importance to decide whether 
the sac contains nerve structures or fluid only. An explo- 
ratory puncture with a needle (attached to an electric bat- 
tery) will help to clear up the point. 

The course and prognosis of spina bifida will vary much 
according to the extent of the hernia ; in some instances, 
the sac ruptures before or very soon after birth ; these 
cases are invariably fatal. The danger of rupture with 
subsequent purulent meningitis is great in all cases. If the 
opening of the sac is closed, the child may continue to live 
for a long period, but it is after all rare for a person with 
spina bifida to live to middle life. If the sac contains nerve 
structures, the prognosis is less favorable than if it contains 
fluid only. 

Treatment. — Much ingenuity has been exercised by 
surgeons in the attempt to cure these cases. Compres- 
sion and ligature (of the pedunculated tumor) have been 
practised in former years, but the results have been disap- 
pointing. Ahlfeld was probably the first to cure a case of 
this sort by surgical procedure. Of late years surgeons 
have become much bolder. Bayer and Hildebrand have 
reported a number of successful cases. For the details of 
these operations the reader is referred to the publications of 
these surgeons (see Bibliography) and to the works on sur- 
gery. It is sufficient to add that both the meningoceles 
and the meningo-myeloceles are considered fit cases for 
operation if the child's health is good, and if there are no 
serious complications such as hydrocephalus, deformities 
of the extremities, ectopia of the bladder and the like. In 
case the exact contents of the sac are in doubt, an inci- 
sion is justified, and free incision of the sac is always to 



444 THE NERVOUS DISEASES OF CHILDREN. 

be preferred to injections into it of iodine and similar 
fluids. 

It is not safe to perform any operation until several 
months after birth. 



BIBLIOGRAPHY. 

Ahlfeld: Deutsche Med. Wochenschrift, 1879. No. 44. 

Bayer: Prager Med. Wochenschr. , 1889, 1890, 1892. 

Furst: Gerhardt's Handbuch, p. 333. (Contains literature to 1880.) 

Von Gieson : Heterotopia, etc. New York Medical Journal, 1892. Sept 

24, Oct. 1, Oct. 15. 
Kronthal : (Heterotopia) Neurolog. Centralbl., 1892, p. 730. 
Kahler u. Pick: Prager Vierteljahrsschrift, 1879. Neue Folge. 2 Bd. 
McNutt and Post : Keating's Cyclopaedia. 
Marchand : In Eulenburg's Real Encyclopaedic, 1889. 
Muscatello: Arch. f. Klin. Chirurgie. Bd. XLVII. 
v. Recklinghausen : Virchow's Arch., Bd. CV., p. 243. 
Remak : Berliner Kl. Wochenschr., 1888, p. 509. 
Sutton: Lancet, Feb. 25, 1888. 

Tooth : (Heterotopia) British Medical Journal, 1891, April 11, p. 803. 
Turner: (Heterotopia) British Medical Journal, 1891. 
Virchow : Virchow's Archiv, Bd. XXVII. 1863. 



DISEASES OF THE BRAIN. 
CHAPTER XXV. 

ANATOMY, PHYSIOLOGY, AND PATHOLOGY OF THE BRAIN. 

The study of the diseases of the brain must be preceded 
by a short discussion of its structures and functions as well 
as of the morbid processes most frequently affecting it. 
This discussion can in no sense be complete, as the author 
will simply endeavor to give a short review of such well- 
established facts as have a distinct bearing upon clinical 
diagnosis.* 

It is unnecessary to enter upon a detailed account of the 
configuration of the surface of the hemispheres. The names 
of fissures and convolutions and their relation to the skull 
can be gleaned from the annexed cuts. Not much more 
than thirty years ago the cortex of the brain was supposed 
to be a homogeneous mass, both as regards structure and 
function. Broca was among the first (in 1862) to show that 
the left third frontal convolution was the centre for speech ; 
a little later Hughlings Jackson described partial epilepsies 
of cortical origin which were in direct contradiction of the 
supposed homogeneous character of all cortical areas ; but 
the doctrine of a strict localization of function in the hemi- 
spheres was not fairly launched until Fritsch and Hitzig 
proved by experiments upon animals that movements of 
different parts of the body could be excited by electrical 
stimulation of definite areas of the cortex. 

The experiments of Ferrier, Munck, and Goltz (the last-named an oppo- 
nent of extreme localization), and the clinical studies of Broca. Jackson. 

« 

*The reader who is anxious to gain a fuller knowledge of the anatomy of the brain 
is referred to the text-books of Gowers, Gray, and Dana ; to the excellent lectures of 
Edinger on the structure of the central nervous system, and to Schaefer's revised edi- 
tion of that part of Quain's Anatomy which treats of the nervous system. 



446 



THE NERVOUS DISEASES OF CHILDREN. 




Fig. 119. — Right Hemisphere of a Simply Convoluted Brain. (Schafer.) Sulci — Ro., 
Rolandic or central ; g, its superior genu ; Sy. a., anterior limb of Sylvian (x, as- 
cending part, y, horizontal part) ; Sy.p., posterior limb of Sylvian ; Sy. p. asc, as- 
cending ramus of posterior limb ; f u superior frontal ; / 2 , inferior frontal ; / 3 , middle 
frontal ; f 4 , paramesial frontal ; d, diagonal, placed in this instance rather low down, 
and communicating with the Sylvian; p.c. inf., inferior precentral ; p.c. i. ant., 
its anterior ramus ; p.c. sup., superior precentral ; p.c. m., mesial precentral ; p.c. 
tr., transverse precentral ; rtc. tr., transverse retro-central; i.-p. inf., intra-parietal, 
pars inferior (inferior postcentral) ; i.-p. sup. , intra-parietal, pars superior (superior 
postcentral) ; i.-p. post. s. hor.^ intra-parietal, pars posterior seu horizontalis ; i.-p. 
post., intra-parietal, pars posterior (paroccipital of Wilder) ; i.-p.pr. asc, an ascend- 
ing branch of the intra-parietal ; p.-o. , parieto-occipital ; occ. atit., anterior occipital ; 
occ. /at., lateral occipital ; calc. , posterior end of calcarine ; t x , first temporal or par- 
allel ; /j asc, its posterior ascending extremity, detached; h, second temporal; t 2 
asc, its posterior ascending extremity joined to and apparently continuous with the 
first temporal. 

Gyri — Fi, F 2 , E 3 , first, second, and third (superior, middle, and inferior) frontal ; 
a, posterior part of third frontal ; b, middle part (pars triangularis) ; c, orbital part ; 
A. F., ascending frontal ; A. P., ascending parietal ; T lt Ti, T 3 , first, second, and 
third temporal. 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. A.A.J 




Fig. 120. — Mesial Aspect of the Left Hemisphere of a Simply Convoluted Brain. 
(Schafer.) Sulci — Ro. , upper end of Rolandic ; p. c. m., mesial precentral ; f 5 , me- 
sial frontal ; c. m., calloso-marginal ; pr. I., prelimbic (anterior end of calloso-margi- 
nal) ; pr. 1. asc, an ascending branch of the prelimbic ; paracentr., paracentral (pos- 
terior end of calloso-marginal) ; p.-L, post-limbic ; ro., rostral ; ro. inf., inferior ros- 
tral; p.-o., parieto-occipital ; calc. ant., stem of calcarine ; calc. post., posterior part 
of calcarine ; i, 2, 3, 4, places where annectent gyri occur in calcarine and parieto- 
occipital fissures ; t 3 , third temporal ; coll., collateral or fourth temporal ; //. (placed 
on the fascia dentata) has the hippocampal fissure just below it. 

Gyri — F x , marginal part of first frontal ; C, callosal (gyrus fornicatus) ; H., hippo- 
campal ; unc. , its uncus; h. , dentate; T it fourth temporal (fusiform lobule); 7^,, 
fifth temporal or infracalcarine (lingual lobule). 

c. c, corpus callosum ; spl., its splenium ; g. , its genu ; r., its rostrum ; /v., for- 
nix ; _/?., fimbria. 



44^ THE NERVOUS DISEASES OE CHILDREN. 

Charcot, Nothnagel, Franck, Pitres, Bernhardt, Wernicke, Horsley, Seguin, 
Starr, and many others, have helped us to assign definite functions to distinct 
areas of the brain. Some of the points may need slight revision, a few are 
still in doubt, but in the main the centres for motion, for sensation, for speech, 
for sight, and for hearing have been established for all time. In the an- 
nexed figures (p. 449) the facts as they have been ascertained are stated. 

It is necessary to be able to map out on the head the areas of the skull 
corresponding to the various centres in the cortex. A number of different 
methods have been suggested by Reid, Horsley, Hammond, and others. The 
" guiding lines " of Reid have proved the most serviceable. (Fig. 123.) The 
motor area is the one most frequently exposed by surgeons. To find this area 
we must locate the upper end of the fissure of Rolando. This can be done by 




Fig. 121.— Relation of Brain to Skull Lines. (Ecker.) F, frontal lobe ; P and P 2 , 
parietal lobe ; O, occipital lobe ; T, temporal lobe ; Cb, cerebellum ; C, central 
fissure ; A and B, anterior and posterior central convolution ; S. S., fissure of Syl- 
vius. The cranial sutures will be recognized easily. 

drawing a line from the root of the nose over the top of the head to the occip- 
ital protuberance. A point about three-fourths of an inch back of the middle 
point of this line corresponds to the upper end of this fissure, and the fissure 
forms an angle of sixty-seven degrees with the median line. Several instru- 
ments (the author has used Wilson's cyrtometer) have been constructed, of a 
horizontal and a vertical strip of metal, joining each other at this angle. The 
vertical strip can be so adjusted as to cover the fissure of Rolando, and if the 
vertical strip is of the same length as the central fissure (about three and a 
half inches), a division into three equal parts will give the three chief divisions 
of the motor area, each area extending about an inch to each side of the 
central fissure. The fissure of Sylvius — the lower boundary of the motor 
area — can be mapped out as follows : Draw a line from the lower margin of 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



449 



the orbit to the auditory meatus. Draw a second line parallel to the first, 
from the external angular process of the frontal bone, and let it extend back 
one inch and a quarter. This is the " first point." From the parietal emi- 
nence draw a perpendicular line to the base line ; on this line, three-fourths 




Fig. 122. — Diagrams representing the Localization of Cortical Centres. (Dana.) Those 
assigned to the frontal lobes the author considers doubtful ; also the distribution of 
motor centres on the median surface beyond the paracentral lobule. 



of an inch below the eminence, note " point two." A line joining these two 
points indicates the position of the fissure of Sylvius. This fissure is about 
four inches long. If we prolong the line of the fissure of Sylvius to the me- 
dian line we get the position of the parieto-occipital fissure at the junction of 
29 



450 



THE NERVOUS DISEASES OF CHILDREX. 



the two lines. Most of the centres can be located with reference to these 
three fissures. (For some further details see chapter on Abscess, following 
Ear Disease.) 

In locating the motor areas the author has been in the habit of verifying 
the correctness of these lines by making an electrical test (with a weak fara- 
dic current and using Keen's electrode) of the centre exposed before the 
dura is opened. If the centre has been carefully located, irritation of the ex- 
posed dura will cause contractions of the parts represented in such an area. 
This method is of the greatest service in directing the surgeon as to the 
further enlargement of the opening in the skull. 




Fig. 123 — A, Peid's Lines 



B, Relations of the Fissures and Convolutions to the 
Guiding Lines. 



In the various centres of the cortex, the cells may be 
considered to be the most important structures. These are 
connected with the cells in other convolutions and with the 
distant parts of the central nervous system by elaborate 
systems of fibres (association, commissural, and projection 
fibres). Impulses from and to the hemispheres are conveyed 
by definite pathways or tracts. The exact course of these 
we should be able to trace from the cortex to the periphery, 
and from the peripheral organs inward. Let us begin with 

The Motor Tract. — On the convexity the motor area 
is bounded by the pre-central and post-central fissures and 
includes the fissure of Rolando ; it extends downward to the 
fissure of Sylvius, and a little forward, including the third 
frontal convolution. In the anterior and posterior central 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



45 



convolutions the motor fibres are so distributed that the 
upper third represents the leg centre, the middle third the 
arm centre, the lower third the face centre, while the third 



Leg 



Arm 



Face 




Fig. 124.— Motor Fibres for the Facial Nerve and the Extremities Horizontal section 
through the hemispheres, crura, pons, medulla oblongata, and spinal cord. (Ed- 
inger.) 

frontal convolution is the centre for motor speech innerva- 
tion.* The motor area of the brain includes the paracen- 
tral lobule of the median surface. (Fig. 124.") 

* Further subdivisions of the motor area have boon determined by Horsley and 
others in monkeys, and I have found by electrical stimulation of the adult brain, ex- 



452 



THE NERVOUS DISEASES OF CHILDREN. 



The fibres which come from these various motor centres, 
and which constitute only a part of that large mass of 
white fibres known as the corona radiata, pass from the cor- 
tex of the brain downward through the centrum ovale to 
the region of the large ganglia. In order to reach the 
lower cerebral and spinal portions, they are compelled to 
penetrate the ganglia. Thus the entire tract of white fibres, 
including those that have to do with motion, passes first be- 
tween the caudate nucleus and the lenticular nucleus, and a 

little farther downward be- 
tween the optic thalamus 
and the lenticular nucleus. 
This tract of w r hite fibres, 
bounded by the gray gan- 
glia, is called the internal 
capsule, in contradistinction 
to the external capsule, so 
called by the older writers 
— a band of white fibres 
lying on the outer side of 
the lenticular nucleus, but 
having very little clinical 
interest. 

In the internal capsule 
the fibres coming from the 
leg, arm, and face centres 
interlace in such a manner 
(Fig. 125), that the face 
fibres lie innermost, the arm 
fibres occupy a median po- 
sition, and the leg fibres lie 
externally to the other two. 
In the internal capsule we 
have, in addition, fibres from the frontal portion of the 
brain to the thalamus, and from the frontal portion of the 
brain to the pons. It contains also tracts connecting the 
nuclei of the cranial nerves, and all of the fibres of general 
and special sensation, w r ith the cortex. 




Fig. 125.— Diagram to show the Relative 
Position of the Several Motor Tracts in 
Their Course from the Cortex to the 
Crus. C. N., caudate nucleus ; Th. O., 
optic thalamus. To the right of the cau- 
date nucleus is the lenticular nucleus ; 
between them, the internal capsule ; be- 
low the optic thalamus, and the crus. 
(Gowers.) 



posed by the surgeon in cases of epilepsy, that the centres for various movements are 
quite as distinct in the brain of man as in that of the lower animals. 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



453 



The motor fibres as they emerge from the region of the 
internal capsule pass into the crus cerebri, occupying in this 
portion of the brain a position very near the ventral sur- 
face. This position the motor tract retains on its down- 
ward path through the pons and the medulla. In the lower 
portion of the medulla the motor fibres decussate with the 
motor tract of the other half of the brain, and the greater 
part of the motor or pyramidal 
tract passes into the lateral col- 
umns of the opposite half of the 
cord, while a small portion of its 
fibres does not decussate and 
passes downward on the same 
side in the anterior columns of 
white fibres in the cord. At each 
level of the spinal cord a connec- 
tion is established between the 
pyramidal tracts in the lateral and 
anterior columns and the gray 
matter in the anterior horns of 
the spinal cord. The ganglion 
cells in this gray matter connect 
with the anterior nerve - roots ; 
these pass through the spinal cov- 
erings, after joining with the sen- 
sory fibres which come from the 
posterior portion of the cord, and 
together with these and with sim- 





iliar fibres from other segments of 



Fig. 126. — Diagram representing 
Motor Innervation of a Muscle, 
and the Two Divisions of the 
Motor Tract. (After a figure 
from Edinger.) 



the cord, form the various nerve- 
plexuses from which the nerve- 
trunk issues. 

Every portion of this motor tract, from the cortex of 
the brain to the peripheral muscles, has been thoroughly 
studied and its exact location firmly established. We can 
divide the motor path for any one muscle of the body 
into two distinct parts. This is not an anatomical ca- 
price but a division of the utmost importance from a purely 
clinical point of view. The first division extends from 
the cortex of the brain through the lateral columns of the 



454 THE NERVOUS DISEASES OF CHILDREN. 

cord to the ganglion cells of the anterior horns ; the sec- 
ond division from the same ganglion cells to the periphery. 
The first attempt in the way of localization of a lesion caus- 
ing a paralysis should be to determine whether the symp- 
toms point to a lesion in Division I. or in Division II. of 
the motor tract. (Fig. 126.) A lesion anywhere in Part I. 
gives rise to spastic paralysis (without atrophy) and to in- 
creased reflexes, but the electrical reactions of the para- 
lyzed parts remain entirely normal. A lesion anywhere in 
Division II. causes an atrophic and flaccid paralysis; the re- 
flexes instead of being increased are diminished, the electri- 
cal reactions, far from being normal, are distinctly altered. 
(For an explanation of these facts see page 279.) 

To determine more accurately in what part of the first 
or second division of the motor tract a lesion may be which 
gives rise to a definite set of symptoms other facts must be 
considered. 

Starting with the cortex, it is very evident from an ex- 
amination of Fig. 124 that the motor fibres in the cortex 
are spread over a considerable area, whereas in the internal 
capsule the fibres are closely packed together. The result 
of this peculiar distribution of the motor fibres is that a 
lesion, even of tolerable size, in the cortex may give rise to 
a paralysis of that part only which is represented by the 
area affected. Thus monoplegia (paralysis of a single mem- 
ber) may be due to a lesion of the cortex, and is never the 
result of lesion in the internal capsule ; but if the lesion is 
large enough to cover the entire motor area in the cortex 
a hemiplegia of the opposite half of the body may result, 
just as it results from a relatively small lesion in the inter- 
nal capsule ; there must, however, be some agreement be- 
tween the size of the lesion and the character of the symp- 
toms. We may infer that if a hemiplegia is due to a lesion 
in the cortex there will be very marked symptoms of cere- 
bral disease (coma, convulsions, etc.), the disturbance of 
brain function will be very great, whereas a like paralysis 
may result from a lesion in the internal capsule with but 
little or no loss of consciousness and without convulsions. 

There is still another symptom which points directly to 
the cortex as the seat of the trouble. It is the special prop- 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 455 

erty of the motor area of the cortex to respond to any le- 
sion that is not absolutely destructive by convulsive seizures, 
and these convulsive seizures are restricted to the parts 
governed by the area affected (Jacksonian epilepsy). If, 
therefore, hemiplegia is associated with localized convul- 
sions the probability is very largely in favor of a lesion in 
the cortex. But every interference with the general action 
of the brain leads to convulsive seizures, and so we find that 
even in cases of lesion of the internal capsule convulsions 
may occur, but these convulsions are rarely of the Jack- 
sonian type, and are rarely or never repeated. No infer- 
ence can safely be made from initial or single convulsions, 
but a repetition of convulsions is thoroughly characteristic 
of cortical lesions. 

Cortical lesions occur relatively more frequently in children than in adults. 
In view of the recent progress in cerebral surgery it is absolutely necessary 
that every physician should be able to determine whether a lesion is in or 
near the cortex, in which case it may be reached by the surgeon's knife, 
or whether it is in the interior of the brain, for if so surgical interference 
is not warranted. The following table will give the characteristic symptoms 
of lesion in the cortex and in the internal capsule : 

Symptoms of Cortical Le- Symptoms of Lesions in the In- 
sions. ternal capsule. 

Monoplegia, possibly hemiplegia. Always hemiplegia. 

If hemiplegia, lesion must be large, General brain disturbances may be 

and general brain symptoms cor- slight, 
respondingly great. 

Jacksonian epilepsy ; repeated con- Initial convulsions may occur, but a 

vulsions. repetition of convulsions very rare 

and never of Jacksonian type. 

General symptoms of lesion of Divi- General symptoms of lesion of Divi- 
sion I., in motor tract. sion I., in motor tract. 

If the lesion is anywhere in the motor tract below the 
point of emergence from the internal capsule the symptoms 
caused by it are characterized by the association of paral- 
ysis of various cranial nerves, with a hemiplegia of the op- 
posite half of the body, thus giving rise to what is known 
as alternate or crossed hemiplegia. A lesion in the crus is 
easily recognized by the association of oculo-motor symp- 
toms (ptosis and complete paralysis of the internal ami ex- 



456 



THE NERVOUS DISEASES OF CHILDREN. 



teral muscles of the eye, excepting the rectus externus), 
with paralysis of the opposite half of the body. The in- 
dependent paralysis of the fourth nerve, which is also sit- 
uated in this vicinity, is very rare. I have not yet seen 
any case of paralysis in the adult or in a child in which 




Fig. 127. — View from Before of the Medulla Oblongata, Pons, Crura Cerebri, and 
Other Structures. (Thomson.) The numbers refer to the cranial nerves. 



paralysis of the superior oblique only was associated with 
paralysis of the opposite half of the body. As we follow 
the motor tract lower down, the symptoms become still 
more complicated. We have next to consider lesions in 
the pons. 

The pons and the medulla contain, in addition to other 



ANATOMY AND PHYSIOLOGY OF THE BRAIN 457 

structures, the nuclei of the fifth to the twelfth cranial 
nerves ; the result is that disease of these parts is character- 
ized by symptoms pointing exclusively to the nuclei, or by a 
paralysis of the extremities associated with various cranial 
nerve palsies. The rule is an alternate hemiplegia with 
paralysis of one or more of the lower cranial nerves of the 
same side of the body as the lesion. We have to note, 
however, one important exception to the rule that cerebral 
paralysis is always hemiplegic. In the pons, and particularly 
in the medulla, the pyramidal tracts are so close together 
that a lesion of moderate size (a tumor, a hemorrhage, or a 
patch of meningeal thickening) may involve both motor 
tracts, thus bringing about a paralysis of all four extremi- 
ties. This is common in cases of specific meningitis of the 
base. Generally a unilateral palsy is first developed, to- 
gether with paralysis of one of the lower nerves (VII- 
XII.), but by degrees this paralysis spreads from one side 
of the body to the other, so that finally all the extremities 
are completely paralyzed. (Fig. 127 and Fig. 3, Plate I.) 

This peculiar association of symptoms, that is, cranial nerve palsies with 
alternate or double hemiplegia, is so unique a combination that one can 
scarcely fail to make a proper diagnosis, at least as regards the site of the 
trouble. In spite of the accuracy of our knowledge, however, there is many 
a pitfall, and as a matter of fact few lesions are more difficult to diagnosti- 
cate than those in the upper portion of the pons. In this part of the brain 
the central fibres connecting the facial nuclei with the cortex have not yet 
decussated, so that a lesion in one-half of the upper portion of the pons 
will naturally produce paralysis of the face and both extremities in the 
opposite half of the body. This paralysis can scarcely be distinguished 
from that which results from a lesion in the internal capsule unless an af- 
fection of some of the other nerves in this vicinity, the sixth or the 
eighth nerve for instance, points in addition to disease of the caudal por- 
tion of the pons. Lesions in the lower half of the pons are easily recognized 
by the combination of facial palsy of the same side as the lesion with 
paralysis of the opposite half of the body. This paralysis can be distin- 
guished, moreover, from a palsy due to a lesion higher up in the brain by the 
paralysis of every branch, and not merely of the lower branches, as in the case 
of internal capsule lesion. Disease of the facial nuclei or of the facial nerve- 
roots may cause changes in the electrical reactions, which are never present 
in capsular or cortical lesions. Other cranial nerves are also frequently in- 
volved in disease of the pons. On careful examination the various func- 
tions of the trigeminal nerve may be found impaired, and a weakness or 



45§ THE NERVOUS DISEASES OF CHILDREN. 

paralysis of the rectus externus, of one or both eyes, may point to involve- 
ment of the sixth nerve ; the trigeminal and abducens symptoms will be on 
the side opposite to the paralysis of the extremities. If the disease is a lit- 
tle more extensive the eighth nerve will also be affected, and deafness may 
be added to the other symptoms. If the disease is limited to the medulla the 
fifth, sixth, seventh, and eighth nerves will escape, while the ninth, tenth, 
eleventh, and twelfth nerves will be more or less involved. Of these the 
hypoglossal is more frequently affected, as is shown by paralysis and atrophy 
of one or both halves of the tongue ; by paralysis of the soft palate ; while, on 
the other hand, disturbances of taste, in deglutition, in articulation (dysarth- 
ria), indicate disease of the ninth, tenth, and twelfth nerves. As the central 
tracts connecting these nerve nuclei with the higher centres undergo decus- 
sation we may have the typical alternate hemiplegia, or, as was explained 
above, we may have paralysis of these various nerves associated with more 
or less complete paraplegia of both upper and lower extremities. The pons 
and medulla contain important reflex centres, so that disease of this region 
is very often characterized by irregularities in the heart's action, and irreg- 
ularities of respiration ; here are also the lower convulsive centres, and ep- 
ilepsy is more frequent, therefore, in disease of this region, than of any other 
part of the brain excepting the cortex. Vertigo, vomiting, ataxia, are also 
more thoroughly characteristic of disease of the pons and medulla than of 
other parts of the brain, for which the proximity to the cerebellum is in part 
to blame. 

The pyramidal tracts in the lateral columns of the cord are, properly 
speaking, a continuation of the cerebral motor tract. The symptoms due to 
disease in these parts has been discussed in a previous chapter. 

The Sensory Tract. — The anatomy of this tract has 
been carefully studied within the last few years. Its entire 
course is not yet as well determined as that of the motor 
tract is ; but one gap after the other is being rapidly bridged 
over. The sensory tract is more complex, too, than the 
motor ; while motion represents a simple form of nervous 
force, sensation may be divided into four distinct manifes- 
tations : the sense of touch, of pain, of heat and cold, and, for 
lack of a better word, though it is by no means adequate — 
the muscular sense. It is very probable that each one of 
these special forms of sensation is conducted along a dis- 
tinct set of fibres. A possible exception may be made in the 
case of pain and touch senses, for the former may represent 
merely an intensification of the latter. The dissociation of 
sensation observed in some spinal-cord diseases, and even in 
diseases of the brain, renders this exception somewhat im- 
probable. (See Chapter XIV., pages 274-276.) 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 459 

The course of the sensory tract in the spinal cord has 
been previously described (page 271). We need do no 
more now than to indicate the course by which sensation 
travels from the periphery inward and upward. (See Fig. 
1, Plate I.) 

After the sensory fibres have entered through the posterior roots into the 
substance of the spinal cord, the course of the fibres varies very much. (See Fig. 
70.) A portion of these fibres passes into the posterior gray matter and soon 
comes into contact with cells, these, in turn, connecting with the antero- 
lateral fundamental tracts. Other fibres pass at once into the posterior col- 
umns and ascend in the columns of Burdach and the columns of Goll into 
the nuclei of these tracts in the medulla oblongata. From these nuclei fibres 
pass after decussating (sensory decussation) into the lemniscus of the oppo- 
site side, and here meet with those fibres which have decussated in the spinal 
cord, and have passed upward in the antero-lateral region. These two sets 
of fibres help to build up the lemniscus of the mid-brain and eventually consti- 
tute a most important portion of the sensory tract between the medulla and 
the cortex. It is these fibres that are involved in the secondary degeneration 
that follows upon disease of the columns of Burdach and of the columns of 
Goll in the spinal cord. 

The exact course of the sensory tract, after it leaves the 
mid-brain, is the point most in question ; we only know that 
it has very important relations to the optic thalamus, that it 
passes through the posterior third of the internal capsule, 
and that at this point the fibres conducting general sensa- 
tion, as well as those intrusted with the special senses, lie 
close together, but as soon as they leave the internal cap- 
sule radiate into different parts of the cortex. The researches 
of later years have led to the plausible conclusion that the 
motor and sensory areas are united (Dana) or overlap each 
other, and that there are purely sensory areas in that part 
of the parietal region which adjoins the motor centres. 
This is surely true of the tactile sense ; Starr has reported 
a case in which a lesion in the arm-centre was followed by 
loss of muscular sense, but whether the senses of pain and 
temperature have a similar termination is still undecided. 

The Visual Tract. — The decussation of the fibres of 
the optic nerves in the chiasm is such (Fig. 128") that 
the largest or median bundle of fibres passes from each 
optic nerve into the opposite optic tract. The lateral or 



460 



THE NERVOUS DISEASES OF CHILDREN. 



lesser bundle remains on the same side. In the posterior 
half of the chiasm there is a small bundle (CG\ establishing 
a union between the two optic tracts ; each tract passes 
through the cms, and sends fibres to the external genicu- 




Fig. 128. — Diagrammatic Representation of the Course of the Visual Fibres. (After 
Obersteiner.) R, retina — shaded parts connected with left hemisphere, the other part 
with the right hemisphere ; No, optic nerve ; Ch, chiasm ; Tro, optic tract ; CM, 
Meynert's commissure ; CG, Gudden's commissure ; /, lateral root of optic tract ; m, 
median root of same ; Tho, optic thalamus ; Cgl, corpus geniculatum laterale ; Qa, 
anterior quadrigeminal body ; Bqa, fibres from the anterior quadrigeminal body ; 
RD, direct cortical fibres of optic tract ; Ss, optic radiations ; Co, cortical layer of 
cuneus ; Lm, median portion of lemniscus. 

late body, to the pulvinar of the optic thalamus, and to the 
anterior quadrigeminal body (the connection with the last 
two has been questioned by Henschen). From these three 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 46 1 

ganglionic masses fibres issue which form what are known 
as the optic radiations or the bundle of Gratiolet ; these fibres 
pass through the posterior division of the internal capsule, 
and thence into the occipital lobe. The visual fibres ter- 
minate in the occipital lobe, more especially in the cuneus, 
which Seguin proved some time ago to be the actual centre 
for vision. 

A lesion anywhere in the visual tract between the hemi- 
spheres and the chiasm will cause bilateral homonymous 
hemianopsia (corresponding halves of the visual field are 
defective) ; but other symptoms will be associated with the 
hemianopsia if the lesion is anywhere else but in the hemi- 
spheres. If the disease is in the vicinity of the crus, hemi- 
plegia and hemianopsia will be associated with oculo-motor 
paralysis of the side opposite the paralyzed limbs ; if in the 
thalamus there will be hemianaesthesia as well as hemianop- 
sia. If the lesion involves the visual tract in the internal 
capsule, there will be a combination of hemianaesthesia, 
hemiplegia, and hemianopsia. If the left occipital lobe be 
affected, word-blindness may be associated with hemianop- 
sia. A lesion in the right occipital lobe is characterized by 
hemianopsia and no other localizing symptom. If the symp- 
toms are due to disease in the optic tract or the optic thala- 
mus, the hemianopsia should be associated with complete 
immobility of the pupil if light is thrown upon the blind 
half of the retina (Wernicke's symptom), whereas the pupil 
should react under similar tests if the lesion is between the 
corpora quadrigemina (the reflex light centre) and the hemi- 
spheres. This symptom is difficult to elicit in the adult ; in 
children I have never been able to get it. 



The olfactory tract probably ends in the gyrus uncinatus, and lesions in 
the latter may be followed by unilateral, or bilateral anosmia. The olfactory 
bulb is a rudimentary organ in man, and holds a similar relation to the ol- 
factory fibres that the spinal ganglia do to the fibres of sensation. The exact 
connections of the olfactory tract have not been clearly determined, but it is 
probable that some fibres passing through the anterior commissure connect 
the olfactory fibres with the cortex of the opposite side. 

The auditory tract begins in one division of the eighth nerve, which is 
distributed in the cochlea, utricle, and saccules (the other division of the 
nerve in the semicircular canal has to do with the function of equilibrium and 



462 THE NERVOUS DISEASES OF CHILDREN. 

the sense of space). The auditory fibres can be traced through the lateral 
root of the acoustic to the nuclei in the medulla near the floor of the fourth 
ventricle. Through these nuclei (chiefly the accessory nucleus connected 
with the lateral root) the auditory tract passes by a few fibres into the su- 
perior olive of the opposite side ; from here fibres pass through the lateral 
lemniscus to the posterior corpora quadrigemina, and thence by paths not 
well defined to the first and second temporal convolutions — the centres for 
hearing ; some fibres evidently pass up through the lemniscus and the chief 
part of the sensory tract to the cortex of the temporal lobes. The auditory 
tract has numerous connections with other nerve nuclei and with the cere- 
bellum. 

Each temporal lobe is in all likelihood connected with both auditory tracts, 
so that a lesion in one temporal lobe is never followed by complete deafness 
of either ear. 

The arrangement of the cranial nerve nuclei and the distribution of 
these nerves at the base of the brain are of great practical importance. The 
special functions of the various muscles supplied by them have been noted in 
the first chapter. For a detailed account the student is referred to the larger 
text-books. A few words only in reference to the nuclei of the nerves 
(III., IV., and VI.) governing the ocular muscles. These nuclei lie in the 
gray matter of the floor of the fourth ventricle, and bear the closest resem- 
blance to the nuclei of the spinal nerves in the anterior gray matter of the 
cord. (On the strength of this analogy Wernicke has described a disease 
affecting these nuclei as polio-encephalitis superior.) If all the nuclei are af- 
fected, the total paralysis following is described as ophthalmoplegia externa 
(ophthalmoplegia interna is rarer and refers to paralysis of the iris only). 
Disease of some of the nuclei gives rise to partial ophthalmoplegia. West- 
phal and Edinger have described a large anterior nucleus. The dorsal and 
chief nucleus lies dorsad of the posterior longitudinal fasciculus, and a me- 
dian nucleus nearer the median line. These nuclei represent the various 
muscles governed by the oculo-motor nerves. The order in which the mus- 
cles are represented is as follows, beginning anteriorly : 1 , Ciliary muscle and 
sphincter iridis ; 2, rectus internus ; 3, levator palpebral superioris ; 4, rectus 
inferior ; 5, superior oblique (trochlear nerve) ; 6, rectus externus (abducens 
nerve). 

It is probable that the fibres transmitting the light reflex are connected 
with the anterior nucleus. 

All these nuclei are connected with the optic tract, and also with various 
cortical areas by paths to a great extent still unknown. 

Total paralysis of all the muscles supplied by a cranial nerve points to a 
lesion involving the root of the nerve after its emergence from the brain. 
Tumors at the base and specific meningitis are the most frequent causes ; 
in exceptional cases the root palsies may be partial and simulate nuclear pal- 
sies. 

The chief symptoms due to lesion of the cerebellum will 
be stated in the chapter on Tumors of the Brain. 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



463 



In addition to the preceding review of the relation be- 
tween the clinical symptoms and the structure, as well as 
function of the brain, we may consider a few questions in 
pathology that apply to the entire central nervous system. 
The brain as well as the spinal cord is subject to the same 
morbid processes that affect other organs. Inflammations 
and tumors occur under very much the same conditions 
in the central nervous system 
as in other organs, but hem- 
orrhages, embolism, thrombo- 
sis, and, above all, primary de- 
generations are more common 
in the brain and spinal cord. 
Degeneration may be primary 
or secondary, and both forms 
gradually lead to a sclerosis 
of the nerve-tissues. 

The symptoms of cerebral 
lesions are complicated by 
those of secondary degenera- 
tion, which is set up in any 
conducting tract in the direc- 
tion in which the impulses 
are transmitted. Thus a dis- 
ease anywhere in the motor 
tract, if it be of sufficient in- 
tensity to interfere with the 
functions of the fibres, will 
cause a descending degenera- 
tion from the affected region 
downward to the end of the 
motor tract. Whether the lesion be in the cortex, in the in- 
ternal capsule, or in the pons, a degeneration starts from the 
level of the lesion and to this degeneration we may attribute 
the spastic rigidity and the increased reflexes. Secondary 
changes always extend as far downward as the point at 
Avhich the gray matter is interposed in the course of the tract. 
Thus in the case of a lesion in the internal capsule the degen- 
eration starting from here can be traced through the pyram- 
idal tracts in the brain and in the lateral columns down to 




Fig. 129 — Diagram representing Sec- 
ondary Degeneration of the Pyrami- 
dal Tracts following upon a Lesion in 
the Left Internal Capsule. (Edinger.) 



464 THE NERVOUS DISEASES OF CHILDREN. 

the lowest portion of the spinal cord, but it never passes 
into any of the anterior nerve-roots of the brachial or 
lumbar plexuses, for at this point the gray matter of the an- 
terior horns is interposed, and the nutrition of the fibres 
connected with the gray matter at each level is not affected 
by this degeneration of the white fibres at higher levels. 
A lesion, too, in the spinal cord which involves the lateral 
columns, and particularly the pyramidal tracts included in 
these, will cause a degeneration from that level downward. 
We see this Avell illustrated in the cases of cervical and 
also dorsal myelitis, in which the symptoms pointing to 
changes in the lateral columns are very much the same as 
though the lesion had been in the brain, with the possible 
exception that in the case of spinal-cord disease the symp- 
toms are very much more apt to be bilateral than unilateral. 

THE BLOOD-SUPPLY OF THE BRAIN. 

The question of the blood-supply of the brain demands attentive study, 
for many serious cerebral diseases are connected with accidents and disturb- 
ances of circulation. The problems have been studied chiefly with refer- 
ence to the brain of the adult, and although the child's brain is liable to very 
much the same accidents that befall the brain in advanced years, there are 
some striking differences which have not yet been properly explained, or even 
investigated. I stated some years ago that the study of the vascular condi- 
tions of the infant's brain would yield ample returns to the student who 
would direct his researches particularly to this question. 

While the question of hemorrhages from the cerebral blood-vessels of 
the adult is understood perfectly, hemorrhages occurring in children have not 
yet been assigned to their proper causes. The atheromatous degeneration in 
trje adult, and the formation of miliary aneurisms responsible for apoplexies 
occurring in later life, must have some sort of counterpart in the brain of 
the child. For the present we are compelled to rely upon a fatty degen- 
eration, occurring commonly in children, to explain those very accidents which 
in the adult we attribute to atheromatous changes. There is still another 
difference. In the adult, hemorrhage occurs most frequently in the vicinity 
of the internal capsule and in the distribution of the middle cerebral artery. 
Mendel has shown by carefully conducted experiments that blood-pressure is 
greatest in the ganglionic branches of the middle cerebral artery. In the 
child, however, this region is less frequently affected. By far the larger 
number of accidents in the child occur in the domain of cortical and pial 
vessels : some of these can be explained as the effect of traumatic injury to 
the surface of the skull and the convexity of the brain. But after making 
due allowance for traumatism, hemorrhage from the cortical vessels is much 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



4 6 1 



more frequent in early life than in later years. The more delicate structure 
of these cortical and pial vessels may be in part responsible for this, but the 
question cannot be considered to be definitely settled until more careful ex- 
aminations shall have been made. 

I have thought it best to call attention at once to points wherein the cir- 
culation in the child's brain differs from that in the adult, but to understand 







Fig. 130.— The Arteries at the Base of the Brain. (After Thane and Duret, from 
Schafer.) The posterior cerebral are cut at their origin from the basilar. Central 
arteries (to the basal ganglia) : am, antero-mesial group arising from the anterior 
cerebral ; al, antero-lateral group (middle cerebral) ; pm,pl (on the optic thalamus), 
from the posterior cerebral ; ach, pch, anterior and posterior choroidal arteries. 
Peripheral arteries: 1, 1, inferior internal frontal (ant. cerebr. art.); 2. inf. ext. 
frontal ; 3, ascending frontal ; 4. ascending parietal ; 5, temporoparietal (middle 
cerebr.) ; 6, 7, 8, ant. post, occipital divisions from the post, cerebral arteries. 



the problems satisfactorily we must first study the exact manner in which 
the blood is carried to, and distributed throughout, the brain. 

The external carotid supplies the scalp, the skull, and the dura mater. 
30 



466 THE NERVOUS DISEASES OF CHILDREN. 

The internal carotid and the vertebral arteries supply the brain, the pia 
mater, and the eyes. The left carotid starts from the highest part of the 
arch of the aorta, while the right carotid comes from the innominate artery. 
The left carotid passes in almost a straight line into the brain, and is thus 
the one into which emboli are hurled much more readily than into the right 
carotid, which comes off at a considerable angle. The pressure in the left 
carotid is also very much greater than in the right, which may account for 
the greater frequency of hemorrhage from the cerebral arteries in the left 
hemisphere than from those in the right. There is a difference also between 
the two vertebrals, the left leaving the subclavian artery in an ascending di- 
rection, while the right subclavian comes off horizontally; emboli can, there- 




Fig. 131. -Cortical Distribution of the Middle Cerebral Artery. (Thane and Char- 
cot.) The remainder of the convexity is supplied by the anterior cerebral (frontad 
and mesad) and the posterior cerebral, cent, antero-lateral group of central ar- 
teries ; 1, inf. ext. frontal; 2, ascending frontal; 3, ascending parietal; 4, parieto- 
temporal artery. 

fore, enter into the left vertebral much more readily than into the right, but 
as they both merge into the common basilar artery the difference between 
the frequency of embolism into the left and right branches is not as great as 
in the case of the branches of the middle cerebral artery. From the com- 
mon basilar artery several branches are given off which supply the cerebel- 
lum, the pons, and the medulla, and two branches pass downward which 
merge into the anterior spinal artery. At the upper end of the basilar artery 
the two posterior cerebral arteries come off, which connect by the posterior 
communicating arteries with the branches of the internal carotid. By these 
and by means of the anterior communicating arteries issuing from the an- 
terior cerebrals, the circle of Willis is completed. Of the variations which 
occur in its distribution we need take no account at present, although we can 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



467 



realize that a different condition of circulation is established if the common 
basilar is supplied by a large artery coming from the carotid, as has been 
stated to be occasionally the case. (Duret.) 

The vertebral arteries and their branches are not infrequently the seat of 
disease. Lesions in the posterior cerebral arteries in particular, giving rise to 
crus lesions, are common enough, yet they cannot compare in importance 
with those accidents which occur in the distribution of the internal carotid 
and its branches ; the internal ca- 
rotid divides into the anterior and 
middle cerebral arteries. The chief 
blood-supply of the hemispheres is, 
therefore, insured by the middle 
cerebral, the anterior cerebral, and 
the posterior cerebral arteries. 
From the three largest cerebral 
arteries and from the circle of 
Willis small branches enter the 
brain, supplying the ganglia and 
the white substance of the brain ; 
the large arteries ramify, however, 
over the surface of the brain, and 
supply the cortex and the white 
substance of the hemispheres. The 
lack of anastomosis between the 
central and cortical divisions is a 
fact of greatest importance, for 
if an embolus happens to be thrown 
into one of the terminal branches 
of either system, the possibility of 
collateral blood-supply is practi- 
cally cut off. Heubner claims that 
there are frequent anastomoses 
between the cortical terminal 
branches, but Duret, whose au- 
thority in this matter stands un- 
questioned, does not agree with 
Heubner in this respect. The 
areas supplied by the cortical branches of the three great arteries can best 
be studied by an examination of Figures 130 to 132. It will be seen that 
the middle cerebral artery is by far the most important ; with its numer- 
ous ramifications it supplies the greater part of the convexity of the hemi- 
spheres, including within its distribution the third frontal convolution, the 
pre-central and post-central convolutions, and the angular gyrus as well as 
the first and second temporal convolutions. The superior frontal and the 
anterior two-thirds of the middle frontal, as well as the upper portion of 
the ascending frontal, are supplied by the anterior cerebral. The occipital 
lobe is supplied entirely by the posterior cerebral. The lower portion of 




Fig. 132. 



-Distribution of Blood Supply at 
the Base. (Schafer.) 



468 THE NERVOUS DISEASES OF CHILD REX. 

the temporal lobe which the middle cerebral does not reach is also sup- 
plied by the posterior cerebral. On the median surface the relative impor- 
tance of the arteries is reversed. The whole anterior and upper portion over 
the frontal end, as far back as the parieto-occipital fissure, is supplied by the 
anterior cerebral artery. The cuneus and the occipital temporal convolutions 
are supplied by the posterior cerebral, leaving but a very small portion at the 
anterior end for the middle cerebral. 

Of the parts enveloped by the hemispheres it is well to know that the cor- 
pus callosum is within the domain of the anterior cerebral, the corpus 
striatum is supplied by the middle cerebral artery, the branches of this ar- 
tery passing to this region through the foramina in the anterior perforated 
space. Duret divides these arteries into the lenticular, the lenticulo-striate, 
and the lenticulo-thalamic, thus indicating their respective areas of distribu- 
tion ; the lenticulo-striate, being the most frequent seat of hemorrhage, have 
received from Charcot the additional designation of the " arteries of cerebral 
hemorrhage." 

The anterior portion of the caudate nucleus is supplied by the anterior 
cerebral ; its upper surface receives some small branches from the lateral 
choroidal branches of the posterior cerebral ; the choroidal plexus of the lat- 
eral ventricles receive its blood from the anterior choroid branch of the inter- 
nal carotid. The choroid plexus of the third ventricle is supplied by a 
branch of the posterior cerebral. The velum interpositum receives its blood 
from the branches of the posterior cerebral, and small branches coming off 
from the circle of Willis supply the quadrilateral spaces at the base of the 
brain. 

Before leaving this part of the subject it may be well to mention that corti- 
cal arteries pass through the subdural spaces, enter the subarachnoid, and at 
this level subdivide into smaller branches. They now lie in the meshes of 
the pia mater, and are embedded in the fissures between the convolutions, 
lying more deeply than the small veins which remain upon the surface of the 
pia mater. The cortical arteries for the most part divide up into a plexus 
of capillaries which supply the gray matter, while the larger branches pene- 
trate the gray matter, passing into the white substance underneath, and here 
also divide into a system of capillaries. 

Venous Circulation.— The veins of the convexity of the brain ascend 
and empty into the superior longitudinal sinus. As the blood flows through 
them in opposition to the law of gravitation, and as they enter the longitudi- 
nal sinus their current is in opposition to the current in the sinus. The re- 
sult is a retardation of blood both in the veins and in the sinus, thus explain- 
ing why clots form so frequently within these veins in an enfeebled condition 
of the body. When the body is in an erect position all these difficulties are 
multiplied, and, as Gowers correctly says. " the man-el is that thrombosis is 
not more common than it is." The veins of the ventral surface of the brain 
take a very different course. The anterior enters the sinus cavernosus, the 
middle the petrosal sinus, and the posterior vein empties into the lateral sinus. 
The blood from the ventricles enters the veins of Galen, and that coming 
from the cerebellum is also poured into the veins of Galen, or into the straight 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



469 



sinus in which the veins of Galen end. On the inferior surface of the brain 
the posterior vein empties into the lateral sinus, and the anterior into the oc- 
cipital sinuses. Communication between the various sinuses is tolerably free, 
with the exception of the superior longitudinal. The exact course, and com- 
munications of the various sinuses one with the other, may be gathered from 
Figure 133. 

After the preceding consideration of the manner in which the blood is dis- 
tributed through the brain it will be well to review briefly the disturbances in 




Fig. 133. — The Veins of the Dura Mater. (Heitzman.) 



blood-supply which occur in the brains of children. In most instances these 
are similar to those of the adult ; a few exceptions to this rule were alluded to 
at the beginning of this section, in which it was pointed out that in the child's 
brain disturbances of circulation are more apt to be exhibited on the surface 
of the brain than in the region of the larger ganglia. In every text-book and 
monograph of diseases of the brain much has been made of the irregularities 
in circulation. Great variations have been supposed to be possible, and a con- 
dition of anasmia and hyperemia of the brain has been held responsible for 
much that was due purely to the ignorance of physicians. 



470 THE NERVOUS DISEASES OF CHILDREN. 

The whole subject of disturbances of circulation has been subjected to a 
most careful critical study by Geigel. His results, startling as some of them 
are, will help to clear away much of the superstition that still surrounds this 
subject. The normal conditions and functions of the brain are unquestion- 
ably dependent upon a sufficient supply of oxygen and the removal of carbonic- 
acid gas ; the nerve elements of the brain are, if anything, more sensitive to 
this regular exchange than are the component parts of other organs. While 
much depends upon the character of the blood which passes through the ves- 
sels of the brain, Geigel has shown that the rapidity with which the blood 
passes through the capillaries is the factor of greatest importance. If the blood 
passes through these capillaries at a sufficient and normal rate, the condition 
is termed, according to Geigel, " Eudiaemorrhysis." If there is a diminution in 
the capillary blood-rate, the condition is termed " Adiaemorrhysis. " If the same 
blood-rate is excessive, the condition is one of " Hyperdiaemorrhysis." Geigel 
has furthermore shown that if the arterial pressure remains the same, a diminu- 
tion in vascular tension is followed by a diminution in the capillary blood-rate. 
In other words, there will be a diminution and dilatation of the arterial blood- 
vessels of the brain, and this will not cause a hyperaemic condition, but, as a 
matter of fact, will produce an insufficient blood-supply, a condition of adias- 
morrhysis. On the other hand, if the vascular tension is increased by the 
spastic contraction of the cerebral arteries, as follows for instance upon irri- 
tation of the sympathetic, we shall find an increase in the capillary blood-rate, 
a condition of hyperdiaemorrhysis, not a condition of anaemia. The same 
author has furthermore shown that the capillary blood-rate is dependent much 
more upon the degree of vascular tension than upon the amount of arterial 
pressure. It is very evident from Geigel's investigations that former views 
with regard to the hyperaemia and anaemia were not well founded, and since 
vascular tension is almost entirely a matter of nerve innervation, it is very 
evident that nervous influences have more to do with changes in the blood- 
supply than those factors have which exert an influence merely upon the 
pressure in the larger blood-vessels. 

In the brain of the adult the most marked disturbances of circulation that 
occur are the result of hemorrhage, of thrombosis, or of embolism. As I 
have pointed out in previous writings, there is no reason why embolism 
should not occur in the brain of the child as well as in the adult, for the con- 
ditions predisposing to embolism (rheumatism, scarlet fever, cardiac disease) 
are frequent enough in early life. The occurrence of thromboses, too, can be 
readily understood ; for we must remember that specific conditions are com- 
mon in children and help to explain a certain proportion of the cases of 
thrombosis, and that the conditions of malnutrition (marasmus) caused by 
defects in food assimilation or by exhausting diseases will be found to be a 
sufficient explanation for the remaining cases of thrombosis. It is more diffi- 
cult, however, to explain satisfactorily the frequent occurrence of hemorrhage 
in children. 

Circulatory disturbances of traumatic origin are far more frequent in 
children than in adults. This refers, properly enough, to cases of compres- 
sion of the head during prolonged labor, or by the blades of the forceps in the 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



471 



process of delivery ; but later in life also instances of traumatic injury are not 
infrequent, and under these conditions, whether as the result of a fall or a 
blow, hemorrhage from the meningeal vessels is very much more frequent 
than from the intra-cerebral arteries or veins, and it is not rare at all on the 
post-mortem table to find an entire hemisphere, or even the greater part of 
both hemispheres, covered by a thin, diffuse clot. 

The weight of the brain of a child varies considerably during the first few 
years of life, and the full weight is not attained until about the age of twenty 
years. In the following table (after Boyd and Schafer) the mean weight is 
stated in grammes : 



Children born alive at term . . . 

Under three months 

From three to six months 

From six to twelve months 

From one to two years 

From two to four years 

From four to seven years 

Fom seven to fourteen years . . 
From fourteen to twenty years 



Males. 


Females. 


330 


283 


493 


451 


602 


560 


776 


727 


94i 


843 


i,o95 


990 


1,138 


1,135 


i,3 01 


1,154 


i,374 


1,244 



It will be seen from the table that the mean weight of the female brain is 
less from birth on than that of the male brain. The proportionate weight of 
the brain to that of the body is greater at birth than at any later period. At 
birth it is as 1 to 5.85 ; at the tenth year, 1 to 14. From the tenth to the 
twentieth year it is as 1 to 30 ; after that the general average of 1 to 36. 5 is 
established. A decrease again sets in late in life. The proportionate weight 
of the cerebellum (inclusive of the pons and medulla oblongata) to that of 
the cerebrum is in the adult as 13 to 87. According to Huschke this weight 
in the new-born is as 7 to 93. According to Meynert the lesser weight of the 
cerebellum of the new-born child puts its brain on a par with the lower se- 
ries of mammalian brains. 



SPEECH DEFECTS — APHASIA. 

Speech defects are very frequent in children. They are present not only 
in various organic diseases of the brain, but also in those in which there is 
defective development of a part or of the entire brain. Speech defect due to 
a general mental impairment is designated " dysphrasia." (Kussmaul.) Dis- 
turbances of speech may be associated with idiocy or imbecility as well as 
with many acute or chronic diseases of the brain. They may also be part of 
deaf-mutism. 

Defective Speech Development.— The normal child is expected to 
begin to speak between the ages of one and one and a half years. Precocious 
children often begin a little earlier. It is not unusual to find children at the 
age of nine months saying such simple words as mamma, papa ; but such 



472 THE NERVOUS DISEASES OF CHILDREN. 

speech is not at all significant of unusual mental development later on. It is 
a general belief, though possibly not demonstrated by figures, that girls begin 
to talk a little earlier than boys do. The environment of a child evidently 
has an important influence in this respect. Children who receive close at- 
tention, of whom parents are unusually proud, are practically forced into talk- 
ing earlier than others ; but if a child has not acquired the first articulate 
signs of language before the age of two years there is every reason to sus- 
pect some defect ; though I have known children to be tardy in the acquire- 
ment of speech, and yet in later years to show not the slightest sign of de- 
fective mental or speech development. A late acquisition of speech may be 
a family peculiarity. I have had under treatment several members of a 
family, none of whom began to articulate properly or to acquire the knowl- 
edge of words until the age of five years, but the children were bright in 
other respects. Such cases, however, are quite rare. Preyer, in his excel- 
lent study of his own child, found that it began to articulate a few sounds at 
the age of eleven months, but that in the twelfth and thirteenth months these 
became more distinct and were occasionally combined into words. In the 
tenth and eleventh months the child was able to understand correctly much 
of what was said to it. We shall see later on that this understanding of 
language is quite as important as the ability to utter speech. 

Parents, as a rule, attach very little importance to defective speech before 
the age of two and a half or three years. If the child has not acquired some 
speech at this time the physician's opinion is asked as to the significance of 
the condition and as to its bearing upon the future mental development of 
the child. In by far the largest number of children defective development 
of speech is part and parcel of a general mental defect. Inasmuch as speech 
is the function of special areas of the brain, it would be natural to expect 
that cases would occur in which speech alone was defective without the im- 
pairment of any other cerebral function, but I have not seen a single such 
instance although I have carefully watched for it for years. Not long ago a 
boy, of six years, was brought to me who was said to come of a family that 
acquired speech late in life. He was not able to utter a single word distinct- 
ly, but mumbled a few indistinct sounds which the mother claimed to be able 
to interpret. Otherwise the boy appeared tolerably bright, evidently under- 
stood language well enough, and the mother, herself an intelligent woman, 
claimed that he was fully the equal of any other child of his age barring 
the mere fact of deficient education. I was willing to accept her statement. 
On closer examination it was discovered that the boy exhibited other than 
mere speech defects ; that he was not able to use the scissors properly, , 
not able to handle knife or fork, and that he was entirely ignorant of the 
difference between colors. It is wise, therefore, not to make the diagnosis 
of an exclusive speech defect unless a very careful examination has been 
made. 

Defective speech may be prenatal in origin, but may also be due to diffi- 
culties during labor, or to disease very early in life before the period of speech 
development has begun. In the prenatal cases the condition will be asso- 
ciated with idiocy and imbecility. In the forms acquired at or after birth the 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 473 

speech defect is associated with some form of paralysis, together with idiocy, 
imbecility, or epilepsy. 

These special speech defects will be referred to again in the chapters 
on the diseases with which they are frequently associated. We are now- 
concerned with that form of speech deficiency which is known as aphasia, 
and which by common usage is now restricted to the loss, through disease, 
of speech that has once been acquired. Such aphasia can, therefore, not 
be said to exist in children under one or two years of age, according to the 
time at which the individual child had acquired the partial use of language. 
It was doubted by many whether typical aphasia was a common occurrence in 
children. In his studies, published some years ago, the author showed that 
typical aphasia was a little less frequent in children than in adults, but that it 
was associated with disease of both hemispheres in children, whereas in 
adults it is commonly associated only with disease in the left half of the brain. 

Long before a child begins to speak it has learned to understand lan- 
guage. The names of persons and of things familiar to it have been per- 
ceived over and over again," until it has learned to associate the sound 
memories with the persons or objects they designate. Months after this 
first step toward the understanding of language has been taken the child 
begins to utter words and to repeat what it has heard. The perception of 
words is followed by utterance. All these auditory impressions, including 
those of language, are received and stored up as auditory memories in the 
first temporal convolution. The memories of muscular innervation in ar- 
ticulating words are preserved in the third frontal convolution, where Broca 
has first located what he supposed to be the absolute centre for speech. 
These two centres are situated in the left hemisphere, which has acquired the 
privilege of governing language in all right-handed persons^. Any disturb- 
ance in that part of the brain which has to do with speech, or rather with 
language, for language is more than speech, will be followed by a loss or im- 
pairment of language, which we term aphasia. If the perception of speech, 
the understanding of spoken language, is defective, we describe the condition 
as one of " sensory aphasia." If the defect is not in understanding language, 
but in uttering it, we designate the condition as one of " motor aphasia," and 
the latter form is much more frequent than the former. 

It goes without saying that sensory aphasia will be present if there is 
disturbance between the periphery and the centre in the first temporal con- 
volution ; a motor aphasia will be present if there is disturbance either in the 
third frontal convolution or in any part of the tract connecting- it with the 
peripheral organs of speech. For a long time Broca's motor aphasia was 
supposed to be the only form of speech disturbance, and the third frontal 
convolution was presumed to be the sole centre of spoken language. 

The recognition of sensory aphasia we owe to the studies of Wernicke. 
who in a brilliant monograph on the subject established for all time sensory 
aphasia as the complement of motor aphasia. This convenient division ot 
speech disorders has held full sway ever since, and its details have been 
elaborated by Lichtheim and a host of others. In the main modern authors 
have fallen in completely with this theory. 



474 



THE XERVOUS DISEASES OF CHILDREN. 



In beginning the study of aphasia diagrammatic representations of the 
mechanism of language have been of great benefit. As the child learns to un- 
derstand spoken language, the first auditory impressions are carried by the path 
a A to the centre A, in which the word memories and sound memories 
are stored up. From this centre A a connection is established with the cen- 
tre M, in which the memories of movements necessary to execute articulate 
sounds are stored up, and from this centre M a path passes outward toward 
m, by which impulses travel toward the organs of articulate speech. The 
simple repetition of spoken language can be effected by this tract aAMm, but 
most authors have agreed to suppose that another centre, B (Fig. 134), is 
necessary in order to effect an understanding of language, and this centre B is 
to be connected both with A and M. The question arises at once whether this 
centre B is entirely independent of the other two ; whether it represents a 

single portion of the brain or 
whether it does not more prop- 
erly represent all those areas in 
the brain in which there is a con- 
nection between speech centres 
and speech paths ? This simple 
diagram was not only to repre- 
sent the usual mechanism of 
speech, but, according to Wer- 
nicke and Lichtheim, special dis- 
turbances of speech were to be 
developed according to interfer- 
ence with special parts of this 
entire speech tract. It was Licht- 
heim 's great merit actually to 
prove by clinical histories that 
symptoms in certain cases differed 
according to the site of the lesion 
in the centres themselves, or in the 
paths connecting them. If an interruption occurs in M, the centre of motor- 
speech memories, the person will not be able to speak voluntarily, or will not 
be able to read words, and there will be other defects in connection with read- 
ing and writing, which we shall consider later on, but he will still understand 
what is said and what is written. The defect is purely on the motor side, and 
the disturbance is typical motor aphasia. If the interruption, on the other hand, 
is in A, the subject will not be able to understand what is spoken, but he will 
still have volitional speech, and will be able to write and copy. If the lesion 
is in the tract AM, the subject is able to understand spoken language ; he 
is also able to understand writing, and is able to copy. He furthermore can 
speak voluntarily, but gives incorrect answers, mistaking the words ; in short, 
he has what is known as paraphasia. Although the direct path, AM, has 
been interfered with, some connection is evidently maintained by the path 
ABM. Each centre has a connection with the concept centre B, over which 




Fig. 134. 



-Lichtheim's Diagram to Illustrate 
Aphasia. (See text.) 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 



475 



impulses may travel, but this connection is not as direct as when the path 
AM is passable. 

Speech is made more complex by the introduction of the art of reading 
and of writing. In both of these the visual memories of letters and of com- 
binations of letters constitute the most important feature. As the child learns 
the alphabet the visual image of each letter is deposited in the occipital por- 
tion of the hemispheres V (Fig. 135) ; by the recognition of and the power to 
call up these distinct visual memories of letters the child is able to recognize 



Centfo.1 



Fi $ « «tre * 



\ i'l'sjM.re, 




Fig. 135. — Diagram to represent the Speech Centres and the Association Tracts con- 
necting them. (See text.) The arrows indicate the direction in which impulses 
travel. The association fibres between several of the centres conduct both ways. 



them rapidly, and to put them together in definite combinations which go to 
form words. Later on these combinations, from frequent repetition, are so 
firmly engrafted upon the mind that the child no longer resolves these groups 
of memories into their component parts. Referring to the diagram, we must 
suppose that through the visual tract the impression is carried to the visual 
centre in the hemispheres. This visual centre evidently has a very close con- 
nection with the auditory area, for with the sight of a letter its sound is inti- 
mately associated, and in calling up the memories of letters, or groups of letters, 
an auditory sensation is invariably associated with them. We must, therefore. 
suppose that in reading, an intimate association is formed between the centre 



476 THE NERVOUS DISEASES OE CHILDREN. 

A and the visual centre O (Fig. 134) or V (Fig. 135). In reading to ourselves 
we feel that there is a simultaneous activity of the auditory centre, but in read- 
ing aloud there is activity not only of the centre A, but also of the centre M. 
Thus in reading aloud the impulse travels along the path of vVAMM. In 
writing, the matter becomes still more complicated, for the centre V must be 
associated with the centre W, from which the impulses issue which direct the 
muscular movements in writing. 

As we write without necessarily having an auditory representation of the 
sound, or without calling up the motor image of the sound, mere copying 
may be accomplished by the path vVWw (Fig. 135), but if we write from dic- 
tation the auditory centre plays the principal role, and under these circum- 
stances the path would be along aAVWw. If we attempt furthermore to 
read aloud what we have written, we see that we must add a direct connec- 
tion between W and Mm. (E, Fig. 134, also represents writing centre.) 

It is fair to inquire whether, in our present state of brain localization, it 
would be possible to project these more or less schematic diagrams upon 
the surface of the brain with any degree of accuracy. In Fig. 135 this at- 
tempt has been made, and it will be seen that the auditory centre is placed 
in the first temporal convolution, the motor centre in the third frontal, and the 
visual centre in the angular gyrus bordering upon the occipital lobe. The 
writing centre (W) I have placed in the arm centre, for I cannot conceive that 
it should be anywhere else. It has been claimed that a special writing centre 
is located in the second frontal convolution, but this is based upon insufficient 
evidence. The location of these centres cannot be well disputed, but less can 
be said of the position of the paths connecting these centres. It is not strain- 
ing the point very much, however, to suppose that these tracts are included 
among the vast number of association fibres which have been traced in the 
hemispheres by Meynert, Edinger, and others. It has always seemed proba- 
ble to me that the association tracts between the third frontal and the first tem- 
poral as well as those between the temporal and the occipital lobes could, in ad- 
dition to others, be engaged in the important functions of spoken and written 
language. It is still more difficult to tell the exact course taken by the centripetal 
and centrifugal paths connecting these various centres with the periphery. 
The path Mm in all probability passes through the island of Reil, through the 
internal capsule, through the ventral portion of the crus to the nuclei (in the 
pons and medulla) of those nerves which innervate the muscles and organs 
employed in articulate speech. The path a A includes the auditory nerve to its 
nucleus, and the connections between this and the first temporal convolution, 
but the exact course between the nucleus and the centre has not yet been 
firmly established. The path vV is perhaps best known, as it can be no 
other than the visual tract that passes through the chiasm along the optic 
tract, in the bundle of Gratiolet, and to the centre in the occipital lobe. We 
have not endeavored to locate a concept centre, B, for that centre is evi- 
dently implied in the harmonious action of all the other centres. Every con- 
cept is, in reality, nothing but the sum of our separate sensations which en- 
ables us to call up the various attributes of the special object named. The 
concept of a rose is the sum of the sensory impressions (memories) of smell, 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 477 

of form, of color, and of touch, and all of these form one distinct unit ; but the 
general concept can be aroused from any one of these separate memories. 
Thus the odor alone is sufficient to call up the concept of a rose, though it be 
hidden from sight. 

Wernicke, who was the first to attempt a further differentiation of the 
various forms of aphasia, divides both the motor and sensory forms into cor- 
tical, subcortical, and transcortical aphasia. 

Cortical sensory asphasia is due to a lesion in the centre A (Fig. 135). 
In this form the subject is not able to understand what is said to him, nor 
is he able to repeat language ; but of his own volition he can speak freely, yet 
is apt to confuse words. This is due to the lack of control such as every 
normal person constantly exercises over speech through the auditory cen- 
tre A. In speaking we are always aware whether the word we have wished 
to use has been correctly spoken or not, but this controlling influence is lost 
in this type of aphasia. 

In subcortical sensory aphasia the lesion is in the path aA. The subject 
is not able to understand what is said, nor is he able to repeat words, but he is 
able to speak freely of his own volition, and such speech is entirely normal if 
the auditory centre A is intact. Lichtheim doubted whether this form was a 
true aphasia, for the patient is able to speak perfectly ; but if aphasia denotes 
a disturbance of language, and not merely of speech, it is evident enough that 
this form must be included, for the person so afflicted is unable to use lan- 
guage properly, since he cannot understand what is said to him. 

Transcortical sensory aphasia is due to a lesion in the path AB (Fig. 
134). The subject cannot understand what is said but is able to repeat 
words. In short, he is able to imitate language without having any memory 
or special understanding of language. In speaking voluntarily such a sub- 
ject is apt to show a confusion (paraphasia), since he is not able to control the 
meaning of words he utters. 

Motor aphasia has been similarly subdivided ; thus we have cortical motor 
aphasia. The lesion is in the centre M. The subject understands language 
but is not able to utter it, or at best can say but a few words. He is not 
able to speak, of his own volition, nor to repeat words, nor even to call up the 
sound of words within his own mind. This is by far the most frequent form of 
aphasia met with, and was the kind described by Broca and the older authors. 

Subcortical motor aphasia. The lesion is in the path Mm. The subject 
retains a correct conception of the auditory memories of words, but he is not 
able to speak ; is able, however, to indicate the number of syllables in a 
word, thus showing that the connection between M and A is perfect. 

In transcortical motor aphasia the lesion is in the path BM (Fig. 134). 
The subject is able to repeat language, but is not able to speak of his own 
volition. He has also a perfect understanding of language. 

There is considerable difficulty in differentiating between cortical and sub- 
cortical motor aphasia. It is recognized, however, a little more readily In- 
taking into account written language, which is lost in the cases of purely cor- 
tical motor aphasia, and is present in the subcortical form. 

Wernicke has established another special form, which he terms " conduo 



47S 



THE NERVOUS DISEASES OF CHILDREN. 



tion aphasia," which he supposes to be due to an interruption in the path 
AM. In this case there is no sensory and no motor aphasia, but considerable 
confusion in the use and understanding of words. The condition is one of 
true paraphasia. 

This differentiation of symptoms has been carried still further, and corti- 
cal and subcortical and transcortical forms of alexia (the inability to read) and 
agraphia (the inability to write) have been established, which involves such a 
mass of details that we need not consider the subject in this connection, 
more particularly as alexia and agraphia do not play the part in the aphasia 
of children which they do in the adult ; but the whole subject can be well 
summarized in the following table, which I have constructed after the fashion 
of Leube : 

Forms of Aphasia (Wernicke-Lichtheim) and their Symptoms. 



Form of Aphasia. 


Understanding 
of Language. 


Repeating 
Words. 


Volitional 

Speech. 


Reading. 


Ability to 

Write Under- 

siandingly. 


Sensory Aphasia : 












Cortical 


Lost. 


Lost. 


Present. 


Lost. 


Lost. 


Subcortical . . . 


Lost. 


Lost. 


Present. 


Present. 


Present. 


Transcortical . . 


Lost. 


Present. 


Present. 


Present, but 

w i t h o u t 
under- 
standing. 


Lost. 


Motor Aphasia: 












Cortical 


Present. 


Lost. 


Lost. 


Lost. 


Lost. 


Subcortical. . . . 


Present 


Lost. 


Lost. 


Present, but 
not read- 
ing aloud. 


Present. 


Transcortical. . 


Present. 


Lost. 


Lost. 


Present. 


Lost. 


Conduction 












Aphasia 


Present. 


Lost. 


Present. 


Lost 


Lost. 



The diagrammatic representation of language, as perfected by Wernicke 
and Lichtheim, has done much toward an understanding of aphasia ; but the 
close adherence to diagrams has prevented an appreciation of the psychic 
processes of language. 

The psychological point of view, as developed by Jackson and Bastian, 
and recently adopted by Freud, will, in connection with Wernicke's locali- 
zation theories, lead to a better knowledge of the subject. 

Of the forms of aphasia, as laid down by Wernicke, conduction aphasia, 
or paraphasia, due to an interruption in AM, has been severely criticised by 
Freud. He states very correctly that since language is acquired by the path 
AM, cases should occur in which a person is able to utter words but not to 
repeat words ; such a case has not been found, and we cannot deny the cor- 
rectness of Freud's conclusions that the paths for voluntary speech and for 
repeating words are necessarily identical. 

It is evident, furthermore, than paraphasia is not limited to disease in the 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 479 

tract connecting the centres A and M, but as Starr has shown from a collec- 
tion of cases, paraphasia results from lesions in various parts of the brain. 

A large clinical experience of aphasia also forces upon one the conviction 
that while some cases are illustrative of the forms laid down by Wernicke and 
Lichtheim, there are many more which do not conform to these types and 
cannot be explained according to the usually accepted diagrams. At the bed- 
side we often find a combination of types of aphasia — a fact which need not 
surprise us if we remember that all the cortical centres of speech are within 
the domain of one artery, the middle cerebral ; and a partial or total loss of 
function of one or more centres, and of the tracts connecting them, may fol- 
low upon disturbances of circulation in one of the larger branches. ( See 
Fig. 131.) 

Wernicke and his followers have not attempted by means of their strictly 
anatomical theories to explain the peculiarities of aphasia which have been so 
lucidly stated in Jackson's earlier essays. (I will instance merely the distinc- 
tion between propositional and interjectional language, the partial retention 
of speech and so forth.) Instead of adhering exclusively to a diagrammatic 
representation of speech it will be far wiser for the present to conceive of 
language (not speech merely) as a function of the cortical centres, and of the 
association fibres connecting one with the other. There must necessarily be 
the freest communication between the various centres, and impulses must 
travel along the association fibres to and from all centres (Fig. 135). These 
paths are traversed so frequently in the acquisition and practice of language 
that excitation of one centre arouses those intimately connected with it. The 
sight of a person familiar to us calls up the name associated with him. The 
mention of his name revives to a degree the visual memory of the man. As 
we are accustomed to think with the aid of articulate signs (words), the word 
memories are aroused more easily than any others, and have freer connec- 
tions with all the centres. 

Grashey effected a distinct advance in the doctrines of aphasia by showing 
that much depended upon the character of sensory impressions, and that un- 
less such impressions persisted a sufficient length of time, other centres could 
not be aroused ; in this way we might explain the difficulty some experience 
in calling up the name of an object as soon as it is seen. If one set of mem- 
ories is lost, it is naturally more difficult to arouse the other memories 
commonly connected with it. The sight of a rose is, under normal conditions. 
sufficient to revive the memory of its name ; but if the visual memory is de- 
stroyed, its spoken name might still be recalled by smelling or touching it ; 
the word-memory in this case being excited through association with the 
tactile and olfactory centres. Tempting as the task is, we cannot follow up 
all the intricacies of the subject. It is sufficient to indicate the author's 
belief that in further studies on aphasia the strictly anatomical and the 
psychological theories must be blended with one another, or must at least be 
allowed to supplement each other.* 

* Special significance must be attached to the monograph of Freud, who has shown 
the defects of Wernicke's and Lichtheim's diagrams, and has proved that several forms 



480 THE NERVOUS DISEASES OF CHILD REX. 

Closely associated with aphasia are peculiar cerebral states in which the 
person has evidently lost the memories of objects once familiar to him. He 
may be able to see, yet not remember, what the object is, nor to what use it is 
put. He may see a watch, a rose, a fork, a ring ; the object no longer 
arouses the memories connected with it. All associations between the va- 
rious centres are interrupted. The condition has been described as psychic 
blindness, psychic deafness, etc. To designate the inability to recognize the 
use of an object, the term apraxia has been suggested. This condition is 
almost invariably associated with aphasia and with disease in the left hemi- 
sphere in right-handed persons, and with disease of the right hemisphere in 
the left-handed person. 

The loss of the musical faculty in some, and its preservation in many, 
cases of aphasia is arousing considerable interest at the present time, but it 
is quite foreign to the subject of aphasia in children. 

Occurrence of Aphasia. — Aphasia is rarely the only symptom of brain 
disease. It does at times, however, represent the most striking feature of the 
case. This is true in traumatic lesions of the skull, if the injury involve parts 
of the brain in which the centres are situated. Thus pure motor or sensory 
aphasia has been observed under such conditions. I have seen a little boy, 
aged six, who after a fall down the cellar-stairs, striking the left half of the head, 
was completely speechless for a period of more than two weeks, but under- 
stood everything that was said to him ; in a few weeks speech was gradually 
regained. The seat of injury corresponded accurately to that part of the 
skull which lies over the lower portion of the fissure of Rolando. Pure sen- 
sory aphasia is rare in the adult. I have seen it in children in connec- 
tion with ear disease ; since the first and second temporal convolutions are 
near to the bony parts of the ear it is natural that abscess arising from disease 
of the inner or middle ear should be located in these parts chiefly. In two 
such patients I have been able to make out distinct sensory aphasia ; in the 
one it was entirely uncomplicated, in the other it was associated with mo- 
tor disturbance of speech. In the case of a little boy, aged six, who had been 
operated on for caries of the mastoid, a transitory sensory aphasia occurred 
a few days after the operation, so that the boy was unable to understand 
what was said to him, and although he was able to speak, would invariably 
give incorrect answers to the questions put to him. Both motor and sensory 
aphasias also may occur in connection with brain tumor if the tumor happen 
to involve one or more of the speech centres, and after various forms of 
meningitis ; but it occurs more frequently with hemiplegia than with any 
other condition. I have referred in the earlier part of this chapter to the 
fact that aphasia is associated much more frequently in children than in 
adults with left-sided hemiplegia, showing that in the children the right half 
of the brain has a far more important relation to speech than is the case 
in adults. Speech disturbances in children are on the whole of shorter dura- 

of conduction aphasia which these authors established are due to imperfect action of 
the cortical centres themselves. To establish this point Freud has fallen back upon 
Bastian's theories regarding the excitability of cortical centres in conditions of disease. 



ANATOMY AND PHYSIOLOGY OF THE BRAIN. 48 1 

tion than in the adult, denoting a greater adaptability of other parts of the 
brain (possibly of the right hemisphere) to assume the function of diseased 
centres. 

To test a child for speech defects note — 1, whether it can understand 
sentences addressed to it, the simpler the better ; 2, whether it can recall the 
names of objects shown it; 3, whether it utters the words correctly, or 
whether it misuses the words ; 4, whether it can repeat words. Next test, 5, 
the child's ability to read ; 6, to read aloud ; 7, whether it understands what 
it reads ; 8, ability to write spontaneously on dictation, and to copy written 
or printed words. Lastly, note whether the child recognizes the use of ordi- 
nary objects. 

These tests will be of great service in determining the form of aphasia 
present and the site of the morbid lesion. In children traumatic injuries to 
the brain, abscesses, and tumors may be localized in one or several of the 
speech centres, and may call for surgical interference. 

Stammering or stuttering is a difficulty in articulation, characterized 
chiefly by hasty vocalization. Kussmaul distinguishes between the two con- 
ditions, but most authors consider them to be practically the same. The 
chief difficulty lies in the simultaneous and precise coordination of the vocal 
cords, the lips, and. the tongue, in the utterance of words. The difficulty is in- 
creased by excitement. Many persons learn to control their more or less 
spasmodic speech ; by careful training through special teachers, the difficul- 
ties of speech can often be conquered. Reading aloud slowly seems to be 
the best exercise. 



BIBLIOGRAPHY. 

ANATOMY, ETC. 

(Consult list appended to Chapter XIV.) 

Dana : Journal of Nervous and Mental Disease, 1888, p. 650. 

Duret : Archive de Physiol., 1874 (Blood Supply). 

Geigel : Virchow's Archiv, Bd. CXIX. ; also Monograph, 1890. 

Grashey : Festschrift, Munich, 1892. 

Henschen : Pathologie des Gehirns. Upsala, 1890, 1892. 

Heubner : Centralblatt fur die Med. Wissenschaften, 1872 (Blood Supply). 

Horsley : Philosophical Transactions, 1888. American Journal of the Medical 

Sciences. 
Jackson: Medical Times and Gazette, 1861-63, 1868, 1871, 1874, 1S76. 

British Medical Journal, May, 1873. 
Lewy : Virchow's Arch., Bd. CXXII. 
Ldwenfeld: Arch. f. Psych., Bd. XVIII. , 1887. 
Marshall : Journal of Anatomy and Physiology, July, 1892. 
Mendel : Neurolog. Centralbl., 1891, No. 24. 
Meynert : Psychiatry: translated by Sachs. 1885. 
Obersteiner : Brain, vol. vii., p. 289 (Blood-vessels, etc.). 
5i 



482 THE NERVOUS DISEASES OF CHILDREN. 

Sachs, H. : Vortrage (Structure of Brain), Breslau, 1893. 
Starr : Journal of Nervous and Mental Disease, July, 1884. American Jour- 
nal of the Medical Sciences, 1884 (Visual Area). 



APHASIA. 

Amidon : New York Medical Journal, 1885. 

Bastian : Brain as an Organ of Mind, International Scientific Series, 1880, 
chapters xxviii., xxix. British Medical Journal, 1887. 

Bernard: Monograph. Paris, 1885. 

Bernhardt : Virchow's Archiv, Bd. CII. 

Charcot: Progr. Medical, 1883. 

Eisenlohr: Deutsche Med. Wochenschr., No. 36, 1889. 

Freud : Zur Auffassung der Aphasien, Leipzig and Vienna, 1891. 

Grashey : Arch. f. Psychiatric Bd. XVI., 1885. 

Jackson : Brain, vols. i. and ii., 1878, 1880. 

Kahler : Prager Med. Wochenschr., Nos. 16 and 17, 1885. 

Kussmaul : Monograph (Ziemssen's Cyclopaedia), 1877. 

Lichtheim : Brain, vol. vii., p. 433, 1885. 

Marie : Revue de Med., 1883, p. 693. 

Ross : On Aphasia. London, -1 887. 

Sachs, B. : Journal of Nervous and Mental Diseases, 1887, p. 503. 

Soltman-Henoch : Festschrift, 1890. 

Starr: Brain, July, 1889. 

Treitel : (Aphasia in Childhood), Volkmann's Samml., 1893, No. 64. 

Watteville, de : Progr. Med., March 21, 1885. 

Wernicke: Der Aphasische Symptomen Complex. Breslau, 1874. Fort- 
schritte der Medicin, 1884, 1885, 1886. See also A Collection of Wer- 
nicke's Essays, Berlin, 1893. 

Westphal : Bed. klinische Wochenschr., 1884. 

Wyllie, J. : Disorders of Speech. Edinburgh, 1894. (Appeared after this 
chapter was completed.) 



CHAPTER XXVI. 

MENINGITIS AND ENCEPHALITIS. 

Inflammation of the coverings of the brain, particu- 
larly of the pia (lepto-meningitis), is at times secondary 
to disease of the brain proper. More often it represents 
the first and most important factor of a morbid process. 
The anatomical designation, meningitis, may be applied 
to a number of different diseases. The symptoms are very 
much the same in all these forms of disease, and such varia- 
tion as occurs depends in part upon the intensity of the 
process, and in part upon the topographical distribution of 
the disease. It is natural, therefore, to expect that the symp- 
toms of a traumatic or of an idiopathic meningitis, which, as 
a rule, involve the convexity of the brain, will be different 
in certain respects from those of a tubercular meningitis, 
which involves the base of the brain ; not forgetting that 
the tubercular process per se is responsible for the severity 
of the symptoms, and the rapid course which that disease 
runs. 

We shall distinguish between — I., Simple acute menin- 
gitis ; II., Tubercular meningitis; III., Epidemic cerebro- 
spinal meningitis, and IV., Meningitis due to various 
causes. 

SIMPLE ACUTE MENINGITIS. 

This form of disease occurs after many infectious dis- 
eases (pneumonia, ulcerative endocaritis, erysipelas, etc.) ; 
after slight traumatic injuries, after insolation, and in con- 
nection with acute nephritis. The symptoms of acute men- 
ingitis are very similar to those observed in all the forms, 
except that they point to an involvement of the convexity 



484 THE NERVOUS DISEASES OF CHILDREN". 

much more frequently than to an involvement of the base. 
The disease begins, as a rule, with a feeling of malaise, with 
vertigo, with nausea and vomiting. These symptoms may 
remit for a few days or even a week, or they may progress 
continuously until the headaches become distressing and 
the vertigo so intense that the child cannot stand, while the 
nausea and vomiting are repeated at frequent intervals. 
The vomiting has in most instances the true cerebral char- 
acter (projectile vomiting). It occurs both after taking 
food and independently of this. If the vomiting under 
these circumstances is associated with a clean tongue, its 
cerebral origin becomes all the more probable. But too 
much stress should not be laid upon this one point, for gas- 
tric disturbance is so common in children, and so often pre- 
cedes the onset of severe cerebral disease that the presence 
of a coated tongue need not suggest the improbability of 
cerebral disease. By degrees the child becomes listless and 
apathetic, it begins to be drowsy, and sleeps a great deal, 
and its existence is taken up with sleeping and crying. A 
convulsive seizure often occurs during this stage. The child 
becomes irritable, restless, shuns the light (photophobia). 
The temperature varies between ioi° and 104 F.; the pulse 
is rapid at first, then becomes irregular and slow ; the 
pupils are contracted for a time and then dilated. The 
child grows more and more unmanageable until the apathy 
deepens, and it finally passes into a comatose condition. 
Before the condition of coma is reached in the vast major- 
ity of the cases slight rigidity of the neck sets in, and the 
upper as well as the lower extremities often exhibit marked 
spasticity. The deep reflexes are, as a rule increased. The 
abdomen is retracted ; the bowels are constipated, and if 
the child is in a deeply comatose condition all efforts at 
feeding it, or at making it take the breast, are entirely un- 
availing. 

According to the severity of the disease these symptoms 
will be developed in a shorter or longer period of time, but 
as a rule a week from the onset is quite sufficient for a 
full display of the symptoms. To those mentioned before 
we may add the loss of vesical and rectal control, the child 
passing urine and faeces into the bed. Vasomotor disturb- 



MENINGITIS. 485 

ances are marked ; taches cerebrales occur in the disease, 
but have no diagnostic significance. In some cases ocu- 
lar palsies (strabismus, ptosis) may be present, and the optic 
neuritis may give rise to temporary or permanent blind- 
ness. A monoplegic or hemiplegic paralysis having all the 
symptoms of a cerebral palsy may be developed. If the dis- 
ease takes an unfavorable turn all the symptoms increase in 
severity, the rigidity of the neck becomes extreme ; opis- 
thotonos is developed ; the blindness continues ; the coma 
is deepened ; respiration becomes irregular, the Cheyne- 
Stokes type becoming more marked until respiration ceases 
altogether. If the patient is to pass on to recovery, the 
symptoms become stationary for a time after the comatose 
condition has been reached ; then the rigidities are les- 
sened, the optic neuritis may recede, and from day to day 
there are evidences of returning consciousness. 

Etiology. — The occurrence of acute meningitis in as- 
sociation with acute infectious diseases will be referred to 
again. " Idiopathic meningitis " has been held by many to 
be a cover for our ignorance. There is no doubt, however, 
that meningitis may be developed without assignable cause 
in a child that comes of healthy parents, and that has itself 
enjoyed perfect health up to the time of the onset of the 
disease. One variety may be attributed to the effect of in- 
tense heat (sunstroke). It is quite likely that a consider- 
able number of the so-called idiopathic forms are due to 
injuries, the traumatic factor being so slight at times that it 
does not receive the attention which it merits. 

Not long since a child was brought to my clinic that had fallen from its 
crib, a fact which the mother mentioned quite casually. When the child was 
first examined it presented the typical symptoms of the first stage of a gen- 
eral convexity meningitis. It was slightly stuporous, unable to stand or sit 
alone, the head was stiff, and it had had occasional spells of vomiting. This 
stuporous condition soon passed into one of deep coma, the child lost ground 
rapidly, never exhibiting, however, any cranial-nerve symptoms. After a pe- 
riod of at least six weeks, during which time the child was more or less som- 
nolent, a favorable change set in, consciousness began to return, and the child 
recovered fully from what was evidently a simple traumatic meningitis. 

Morbid Anatomy and Pathology. — Simple acute 
meningitis is characterized by an inflammatory condition 



486 THE NERVOUS DISEASES OF CHILD REX. 

of the pia mater, which is, as a rule, attended by slight in- 
flammation in the dura and in the gray matter of the brain. 
In contradistinction to other forms this inflammation is of 
a serous, or at least of a non-purulent character. There is 
an increase of cerebrospinal fluid, the arachnoid may ap- 
pear a trifle opaque, while the substance of the brain is dis- 
tinctly oedematous, and even watery. The ventricles are 
distended, and there may be a condition corresponding to 
an acute hydrocephalus. The pia of the convexity of both 
hemispheres is the part most extensively diseased, the pia 
of the base often being entirely free from all disease, though 
a slightly increased exudation of lymph may be apparent 
in the interpeduncular space. 

On microscopical examination the blood-vessels of the 
pia are generally found to be slightly engorged, and an ex- 
travasation of white blood-corpuscles is found in the vicin- 
ity of the blood-vessels. If the disease has lasted, for any 
considerable length of time, the pia and the cortical sub- 
stances have become agglutinated so that the pia cannot 
be removed without tearing the outer layers of gray matter. 

Diagnosis. — The diagnosis of simple acute meningitis 
rests upon the recognition of the symptoms common to all 
forms of meningitis. These are headache, vomiting, coma, 
and convulsions ; irregularity of the pulse and unequal pupils. 
It is well to note also the absence of those factors which 
accompany the graver forms of meningitis (high fever, ba- 
silar symptoms, rapid emaciation, and a rapid increase in 
all the symptoms). The differential diagnosis will not be 
an easy one, and sometimes a positive diagnosis cannot be 
reached until the disease takes an unexpectedly favorable 
turn, or until the patient is seen upon the post-mortem 
table. But the mistake that is most frequently made is that 
the meningeal symptoms accompanying many acute infec- 
tious diseases are at once pronounced to be the symptoms 
of true meningitis. This diagnosis has, to the author's 
knowledge, been made in the first stages of typhoid fever, 
and in measles and scarlet fever beginning with convulsions ; 
the error is common in otitis and in infantile spinal paraly- 
sis that is attended by convulsive seizures and high fever. 
It is well to remember that though these symptoms resem- 



MENINGITIS. 487 

ble those due to meningitis, they are frequently associated 
with other diseases, and that no physician should be in a 
hurry to diagnosticate meningitis, pure and simple, unless 
other diseases can be safely excluded. 

Course and Prognosis. — The course of a simple menin- 
gitis will almost invariably cover a period varying from four 
to twelve weeks, or even longer. In those cases that do not 
tend to recovery the symptoms gradually become intensi- 
fied, respiration becomes more and more difficult, and after 
dragging along for some weeks, now and again yielding a 
hope of recovery, the child finally succumbs from mere ex- 
haustion, from some intercurrent disease, such as bronchitis 
or pneumonia, or from the effects of cystitis, bedsores, and 
the like. 

In those cases that recover after the coma has lasted for 
a period varying from one to three or four weeks, signs of 
returning consciousness are noticed, the child opening its 
eyes, voluntarily looking around, again taking hold of the 
bedclothes, of the hand of the nurse or mother, and so on. 
By degrees the sight improves, hearing, if diminished or in- 
creased, becomes normal, the child begins to take its food 
properly, and from week to week a distinct improvement is 
noticeable, until all the symptoms have disappeared. But 
not all of the cases that recover end thus fortunately. The 
disease does not necessarily kill, but it often leaves distinct 
traces behind. Not a few of those who are permanentlv 
blind owe their misfortune to a meningitis in early vears. 
Permanent paralysis and contractures of one or more ex- 
tremities are attributable to the same cause, and defective 
speech, and, above all, a defective intellect are very often the 
unfortunate outcome of meningitis in early life. Such de- 
formities and defects do not, however, result as frequentlv 
from simple meningitis as from the severer tvpes, from 
which recovery is, on the whole, much rarer. 

Treatment. — In every form of acute meningitis, what- 
ever its origin may be, I am in favor of adopting the fol- 
lowing procedure : 

First, keep the patient absolutely quiet, and in a semi- 
darkened room, and secure the services of a careful and in- 
telligent nurse. Give the child an efficient purgative ; 



THE NERVOUS DISEASES OF CHILDREN. 

none will do better than calomel, which has attained a cer- 
tain dignity in all diseases of the central nervous system. 
Place an ice-bag over the convexity of the skull, or on the 
nape of the neck if the symptoms point to the involvement 
of the basilar portion. Though we cannot claim any direct 
therapeutic effect it will do no harm, for it will at least 
help a little to reduce the general rise of temperature, and 
is useful as the first point of attack in treatment. Place the 
child at once, according to its age, upon moderate doses of 
the bromide and iodide of sodium (three to five grains of 
each every four hours). Give these in simple water, or 
in milk. This treatment may well be persisted in for a few 
days. If no effect is observed, while it is well to continue 
the salts of sodium, other more energetic measures may be 
employed in addition. Among these I would place, first, al- 
ternate lukewarm and cold douching of the nape of the neck 
and the upper portion of the spine, and inunctions of some 
form of mercury, either of the ten per cent, oleate of mer- 
cury or of the unguentum hydrargyri. Inunctions should be 
performed by the nurse or the mother, and done so thor- 
oughly that the mercury disappears into the skin of the pa- 
tient. The combined mercurial and iodide treatment should 
be persisted in until some contraindication arises, or until a 
distinct improvement is noticeable which justifies the hope 
that the child will recover without much additional rigor- 
ous treatment. Under these circumstances the exhibition of 
these drugs may be greatly diminished, and in time stopped 
altogether. 

After the patient has made a fair recovery, the general 
condition of the system will need looking after, and a thor- 
ough course of cod-liver oil, of malt, or of iron, will be quite 
in order, and for some months after recovery from this se- 
vere disease, the child should be kept free from all excite- 
ment, and from all mental and physical fatigue. A restful 
out-of-door life is the very best conclusion to this method of 
treatment. To patients who exhibit the least tendency to 
paralysis or contracture, massage should be given as soon 
as the acute symptoms have disappeared, and electric treat- 
ment should be applied to the parts that are distinctly para- 
lyzed. 



MENINGITIS. 489 



TUBERCULAR MENINGITIS 

Tubercular meningitis is by far the most frequent form 
of meningeal disease in children. It is also the one most 
dreaded, for making a diagnosis of tubercular meningitis is 
almost equivalent to signing the death-warrant of the child. 

The disease itself was first recognized by Robert Whytt, in 1768, and 
since that time has been the subject of innumerable articles ; but every point 
regarding the disease is now so thoroughly known that we can sum up its 
chief characteristics without referring to individual authors. Since Koch's 
discovery of the bacillus of tuberculosis there has been no doubt of the mi- 
crobic origin of tubercular meningitis. Apart from being the expression of a 
general tuberculosis, it has been proved to be caused at times, as in a case of 
Demme, by the direct invasion of tubercle bacilli. 

The disease occurs in children much more frequently 
than in adults ; while it is rare in young infants, it is more 
frequent in children between the ages of six months and 
four years. In families in which there is a distinct heredi- 
tary taint of tuberculosis or scrofula, children that have 
been apparently well are suddenly attacked by this malig- 
nant disease, and healthy children in families in which ev- 
ery hereditary predisposition is wanting, or, at least, denied, 
are also affected. But in the majority of instances the chil- 
dren attacked have been weak and feeble ; many of them 
have suffered from chronic intestinal troubles, from swell- 
ings of the glands, from nasal and aural catarrhs, in short, 
from those conditions from which we may safely argue a 
tubercular infection. A chronic laryngitis or bronchitis, or 
a swelling of the bronchial glands, which may not have 
given rise to any special symptoms, is often the precursor 
of tubercular meningitis. 

Onset. — In contradistinction to other forms of meningi- 
tis, that dependent upon tubercular infection comes on in a 
very insidious fashion. The child first loses its brightness 
and cheerfulness, and complains of an occasional headache, 
and slight nausea ; a vomiting spell may occur. The pulse 
is generally rapid. These complaints almost invariably lead 
to the suspicion of slight gastric disturbance, which, fortu- 
nately, is quite often the case; but every physician will 



490 THE NERVOUS DISEASES OF CHILDREN. 

do well whenever this series of phenomena occurs to be 
watchful, and to give notice that he should be informed if 
the symptoms do not promptly disappear. There may be a 
marked remission or great improvement for a few days ; then 
a change takes place, the nausea becomes more frequent, the 
headache more intense, and the vomiting spells are a daily 
occurrence. In this way a week or more may pass, the 
physician and parents hoping that all the symptoms may 
subside. At the end of about a week little doubt remains of 
the significance of the condition, for if a child is afflicted 
with a tubercular meningitis the headaches become intense 
and persistent, the child gives the short hydrocephalic cry, 
and by degrees becomes somnolent ; slight rigidity of the 
neck is observed, the pulse has become slower, and the 
child shows every sign of serious illness. The further de- 
velopment of the symptoms points to an intense general 
brain disturbance, and to the localization of the disease at the 
base of the brain from which the various cranial nerves issue. 
The temperature is subject to great variations, and during 
the first few weeks it does not, as a rule, rise above 103 F., 
but during the last week it may reach 105° F., and during 
the terminal stages may reach 106 F., or even higher. In 
one case, an hour before death, I recorded a temperature of 
107 F. This is supposed to be due to a paralysis of the 
heat-regulating centres. Respiration is not seriously inter- 
fered with, as a rule, until the child enters upon the ter- 
minal period, during which stage the breathing becomes 
irregular, often of the typical Cheynes - Stokes type, and 
cyanosis is added to the host of other symptoms. 

As we examine the child from head to foot, we are 
apt to find a variety of symptoms. Rigidity of the neck, 
with or without opisthotonos, and excessive painfullness on 
every passive movement of the head or trunk. The major- 
ity of the patients present distinct convergent strabismus, 
due, as a rule, to a paralysis of one or more of the ocular 
muscles. The pupils are unequal and dilated, contracting 
very sluggishly to light, and their reaction during accommo- 
dation can, of course, not be tested in consequence of the 
comatose condition. The conjunctival reflex is lost at an 
early period, and in consequence of diminished movement 



MENINGITIS. 49I 

of the eyelids the cornea becomes cloudy easily. Degluti- 
tion can be carried on only imperfectly, and during an ef- 
fort to open the mouth or to perform chewing motions, a 
trismus is very apt to set in. A further examination of the 
head may reveal a paralysis of some of the branches of the 
facial, the paresis of these muscles being, at times, unilat- 
eral and at other times bilateral ; but the paresis is evi- 
dently due to involvement of the nerve at the base, and if 
an examination is made to bring out this special point, the 
electrical reactions may be found altered in keeping with 
this special localization of the lesion. 

An examination of the eyes will disclose a hyperaemic 
and swollen condition of the papillae. In some instances 
there may be a typical optic neuritis, single or double.* 

The abdomen is retracted, taches cerebrales are easily 
produced (not pathognomonic). 

The upper and lower extremities may be paralyzed to 
a greater or lesser degree ; the exact amount of paralysis 
cannot always be easily determined on account of the en- 
tire absence of voluntary action, and because the physician 
is unable to make satisfactory tests for this special point. 
While the paralysis may be of sufficient interest as illus- 
trating the exact distribution of the meningeal process it is 
of very little practical importance, and loses in value as 
compared with the other and more serious symptoms. The 
paralysis may be hemiplegic; in some forms it is bilateral 
or irregular. The cutaneous reflexes throughout the entire 



*Much has been made of the presence of tubercles in the choroid, and it has been 
claimed by many that the existence of these tubercles is one of the most important 
symptoms of the earlier stages of the disease. The truth of this cannot be gainsaid, 
but as a matter of practical experience it must be admitted that in many cases of un- 
doubted tubercular meningitis the expert oculist does not find tubercles, and that in 
those very cases in which the discovery of such a tubercle would have helped to de- 
termine the exact character of a meningitis, the tubercles cannot be seen, although 
later post-mortem examination leaves no doubt of the tubercular character of the 
process. While we may regard the presence of tubercles, therefore, as a valuable 
corroborative symptom, a failure to detect them should not be allowed to disprove the 
diagnosis if other symptoms would seem to point to the tubercular nature of the 
trouble. Dr. R. L. Randolph has examined thirty-five cases of meningitis of all kinds 
with the ophthalmoscope, and found the fundus normal in seven patients. The optic 
disks were usually congested, with the retinal vessels distended and remarkably tortu- 
ous. In three cases the entire eye was normal, and these three patients recovered. In 
the four fatal cases with normal fundus some other ocular symptoms were present. 



492 THE NERVOUS DISEASES OF CHILDREN. 

body are diminished, often lost. The deep reflexes in the 
upper and lower extremities are, as a rule, increased. 

Epileptiform convulsions are not rare, both at the onset 
of the disease and during later stages. These convulsions 
are, as a rule, general in distribution, and not of the Jack- 
sonian type. If the latter form should prevail, there would 
be good reason to infer that the cortex is diseased as well 
as the base of the brain. Considering the fact of the ex- 
istence of so-called epileptiform centres in the pons and 
medulla, the wonder is not that such convulsions occur, 
but that they do not occur much more frequently. In the 
terminal stages of the disease convulsions become more 
frequent, as they do in many other brain diseases ; and in tu- 
bercular meningitis the frequent occurrence of convulsions, 
with a rise in temperature, may be taken to be the sign of 
the approaching end. In the terminal period, too, the paral- 
ysis becomes complete, the pupils are dilated, the tongue dry 
and furred and the temperature may fall to 93 ° or 94 F., 
until an ante-mortem elevation of temperature begins. 

All the phenomena are remarkably persistent during the 
course of this disease. There are at times slight remissions 
in the ocular palsies, but after the symptoms have once 
been fully developed, they remain very much the same to 
the end. The changes which take place are these : Respira- 
tion is irregular, at times intermittent, the pulse grows 
feebler and slower, deglutition becomes more and more 
difficult, and the child dies from paralysis of the cardiac 
and respiratory centres. 

Morbid Anatomy and Pathology.— Every one who 
has had an opportunity to remove the brain from cases 
of tubercular meningitis is surprised by the few changes 
found in this organ. In the great majority of autopsies, on 
the removal of the calvarium there is very little evidence 
of any active process on the convexity. In some brains the 
pial vessels are much congested, the sinuses are filled with 
clots that have evidently been formed only a few days be- 
fore death, and the hemispheres in general present a more 
or less cedematous appearance. 

Minute tubercles are noticed along the distribution of 
some of the larger pial veins on the convexity. The chief 



MENINGITIS. 493 

changes cannot be noted until the brain has been removed 
from the skull. At the base of the brain the character of 
the disease is easily recognized, the pia is cloudy, and in 
some places bulges out a little from the accumulation of 
fluid underneath. This is particularly noticeable in the in- 
terpeduncular spaces, in which the tubercles are, as a rule, 
most freely developed. These tubercles, often no larger 
than a pin's head, may be scattered throughout the entire 
pia from the optic chiasm to the pons, medulla, and spinal 
cord. The presence of tubercles is not always established 
at the time of the autopsy, but this need not militate 
against the proper diagnosis, for it is a fact, well proven, 
that in undoubted tubercular diseases we may have an in- 
volvement of the pia without tubercles, and occasionally 
the presence of tubercles with but few signs of an inflamma- 
tory process. 

Pathology. — Of the pathology of tubercular meningitis 
little need be said, as it belongs to the order of infectious 
disorders, and the disease is, in almost every instance, a part 
of a general tubercular infection. The tubercular form is, 
moreover, rarely a primary affection, although it sometimes 
occurs in children who have been apparently healthy. A 
thorough, post-mortem examination reveals quite regularly 
distinct foci of disease in the mesenteric or other glands 
from which the infection in all probability took its start. The 
invariably fatal issue must be attributed in part to the gen- 
eral effect of the tubercular poison and its ravages in other 
organs ; for the remarkably slight changes in some of the 
cases that have taken a rapid course are the surprise of 
every pathologist. A few small tubercles, without much 
exudation, even though they be in the vicinity of the pons 
and medulla, can hardly be considered a sufficient cause of 
death, whereas the toxines circulating in the blood may have 
been the initial cause of the paralysis of the vital centres. 

Diagnosis. — The difficulties of a differential diagnosis* 



* The general practitioner is apt to attach too much importance to the general 
symptoms, and too little to the local (basilar) symptoms. The pulse, the tempera- 
ture, the condition of the abdomen, may leave the diagnosis in doubt, but an acute 
ocular palsy, however slight, or an incipient optic neuritis, will indicate the true nat- 
ure of the trouble. 



494 THE NERVOUS DISEASES OF CHILDREN. 

are not limited merely to the period of onset, during which 
time the most experienced physician may well be in doubt 
as to the true character of the disease. But the more diffi- 
cult question arises later on, whether the disease be a simple 
meningitis, whether it be of tubercular origin, or whether 
it represents an epidemic form. In order to determine this, 
it is best to keep in mind the antecedent history of the 
child ; a history of tubercular trouble in the family, or of 
an early scrofulous or chronic catarrhal condition would 
naturally prejudice the physician in favor of a diagnosis of 
tubercular trouble, but everyone has experienced curious 
surprises in this respect, for children of tubercular ances- 
try may have a simple meningitis which is recoverable, or 
they may be afflicted with the epidemic form. I believe it 
therefore to be a good policy to give a rather guarded prog- 
nosis until the general course of the disease, as indicated by 
the symptoms noted above, leaves no doubt of the exact 
character of the meningitis. In addition, it is fairly safe to 
infer that cases of tubercular meningitis invariably present 
basilar symptoms, whereas the symptoms pointing to in- 
volvement of the convexity only occur more frequently in 
the other forms. 

Course and Prognosis. — Tubercular meningitis runs, 
as a rule, a course varying between three and six weeks, 
though the time may be extended a little if the premonitory 
period is taken into account. In the more virulent forms 
death may occur at the end of the first week, or in the 
course of ten or twelve days ; and in these forms I have 
often found only slight post-mortem changes, from which 
we ma}' infer that the general toxine poisoning has been of 
much more consequence than the local deposit at the base 
of the brain. As for the prognosis, no one need hesitate to 
say that it is absolutely bad ; but in making such a prognosis 
the most experienced physician will do well to remember 
that this diagnosis is never quite as certain as is the prog- 
nosis based upon it. There is grave doubt whether cases 
of tubercular meningitis ever recover. 

Henoch, and Rilliet and Barthez, record two cases in which death ensued 
from a second attack, occurring some years after the first ; but even here there 



MENINGITIS. 495 

is room for doubt as to whether the first attack of meningitis was truly tuber- 
cular in character. Pollitzer reports a case of a child which survived three 
years after an attack of basilar meningitis ; at the autopsy he found, at the 
base, the distinct evidences of an old exudation over the pons, which was in 
all probability of a tubercular nature. Freyhan found the tubercle bacilli in 
the cerebro-spinal fluid of a patient who recovered. 

Treatment. — In the treatment of tubercular meningitis 
no time should be lost at the start in resorting to very 
active measures, for the suspicion of the tuberculous charac- 
ter of the process may be unfounded and the patient may be 
fortunate enough to recover. The patient should be put as 
quickly as possible in a quiet, darkened room. Administer 
calomel at once, in a sufficient dose to effect a very copious 
discharge from the bowels. If the child will tolerate it, put 
an ice-bag over the nape of the neck, or if the ice-bag is un- 
pleasant, ordinary cold applications can be tried. As soon 
as the bowels have been moved give the iodides, the bro- 
mides, or mercury, as stated on page 488. 

From the very beginning, too, observe the cardiac and 
respiratory functions, and give mild cardiac stimulants. 
The best are small doses of digitalis and caffeine. 

Special attention should be paid to the feeding of the 
child. Feeding by the spoon and giving the food in very 
small quantities at a time is the only proper method. If 
great care is not exercised the liquids may flow into the 
trachea and produce very uncomfortable symptoms, with 
the possible result of complicating pneumonia. According 
to the condition of the child the physician should exercise 
his judgment, and remember that loss of sleep and lack of 
quiet are often much more harmful than lack of food, and 
every child suffering from any form of meningitis should 
be given ample time for quiet sleep. The old habit of in- 
sisting on half-hourly, or even hourly, feeding is not to be 
commended in these cases. 

Unfortunately there is little reason with the majority of 
patients to change this method of treatment, for whatever 
method be employed the results are equally disastrous; but 
a persistent effort should be made, and the attempt to con- 
quer the disease should not be given up until the child be- 
gins to decline rapidly, until it fails to swallow food, for 



49^ THE NERVOUS DISEASES OF CHILDREN. 

from that time on medication will do very little good. Per- 
sistent administration of medicines, of nutrient enemata, 
and the like, beneficial as they may be in other diseases, in 
these simply tend to prolong the agony of the child, and of 
the careworn parents or relatives. Under such circum- 
stances it is, as a rule, more merciful to exact little in a 
hopeless cause. 

Surgical interference has been attempted in tubercular meningitis. Ord 
and Waterhouse have trephined a case diagnosticated as tubercular menin- 
gitis, and have drained the subarachnoid space ; * the child recovered. There 
is reason to doubt the tubercular character of the disease in this case, but the 
relief after the operation was so marked that the propriety of an operation 
can be entertained before the child is exhausted, if pressure symptoms are 
extreme and the character of the meningitis is doubtful. A recent author 
(Hirschberg) believes that death is due, not to the tubercles, but to intracra- 
nial compression. We have stated on a preceding page that the in- 
crease in intracranial fluid is often very slight indeed. The trephine in Ord's 
patient was applied midway between the external occipital crest and mastoid 
process. The operation has been attempted in this city on an undoubted 
case of tubercular meningitis and the child died very shortly thereafter. 

EPIDEMIC CEREBRO-SPINAL MENINGITIS.f 

This special form of meningitis has attained a sad dis- 
tinction in many countries. In America its ravages have 
been very much greater than in Europe. It is natural that 
American text-books on medicine contain full accounts of 
it, whereas many of the best German and French works 
pass it over lightly. It belongs to the category of infectious 
fevers. The microbic origin of the disease has recently 
been established beyond peradventure, though it seems 
to be still a matter of doubt as to whether one or more 
micro-organisms bear an etiological relation to it. 

Weichselbaum, Ribbert, Eberth, and others have isolated the micrococcus 
lanceolatus from the exudate under the meninges. This same micrococcus 
has also been found in cases of meningitis complicating pneumonia, and in 
meningitis following upon traumatic injuries. Monti claims to have found 

* Such drainage could be effected quite as readily by a lumbar puncture according 
to Quincke's method. 

t Synonyms : " Spotted fever," " cerebro-spinal fever," " typhus petechialis," " fe- 
bris nigra," and " malignant meningitis," are the common synonyms, all of them indi- 
cating the infectious and grave character of the disease. 



MENINGITIS. 497 

the micrococcus lanceolatus in a case of combined arthritis. Klippel found 
the same micro-organism in a patient who died in a demented condition from 
acute meningitis. Flexner and Barker, who have reviewed this subject in a 
recent able article, refer to a case of purulent meningitis examined by Neu- 
man and Schafer in which the pneumococcus and the staphylococcus pyo- 
genes aureus and another fine bacillus were found. Prudden oxamined the 
exudate of traumatic meningitis, in a child, of thirteen months, who had been 
under the observation of Holt; the former isolated the micrococcus lance- 
olatus, and white mice which he inoculated with the organism died in thirty- 
six hours from septicaemia. The inference from all the facts seems to me to be 
that the micrococcus lanceolatus is present in many cases of epidemic cere- 
brospinal meningitis, and also in meningitis complicating other diseases. 
While it is probable, therefore, that it holds an important relation to this dis- 
ease, it can hardly be considered to be the sole factor. The disease may 
possibly be caused by a number of different micrococci. 

The first epidemic of cerebro-spinal meningitis was distinctly recognized 
as such, and well described under the heading of " a malignant non-conta- 
gious fever " by Vieussens in 1805, who described the disease as it appeared 
in Geneva. Thirty-three persons lost their lives during this epidemic ; the 
average duration of the disease was from one-half to five days. A few 
years later epidemics occurred in various parts of Germany, in Holland, and 
in England. Dr. J. Lewis Smith, to whom we are indebted for one of the 
best contributions to our knowledge of this disease, states that the first Amer- 
ican case occurred at Medfield, Mass., in 1806. From 1806 to 1816 it ap- 
peared in various localities both in Canada and in the United States. Be- 
tween 1816 and 1828 one epidemic occurred at Middletown, Conn., and 
another at Vesoul, in France. In 1833 Naples was visited by this epidemic, 
and the disease did not appear again until 1837, and then various localities in 
France were stricken. The military were chiefly affected by the disease, and 
a very large proportion of those affected died from it. Between 1837 and 
1849 France was the chief seat of the epidemic. In the next ten years al- 
most every part of Europe was visited by the disease. In 1 842 another epi- 
demic broke out in the United States, at a distance from the sea-coast, and as 
Dr. Smith says, apparently not by communication from Europe ; but this 
could hardly be maintained with our present views regarding the transmis- 
sion of micro-organisms from one country to another. Epidemics occurred 
in States as widely apart as Alabama and Mississippi, New York and Louis- 
iana. Norway and Sweden were the chief seats of the disease between 1854 
and i860, and since that time scarcely a single city or district has been 
entirely free from the disease. Inasmuch as isolated districts have been af- 
fected, the disease was not supposed to spread in the manner of ordinary 
contagious diseases, but to have been engendered by local conditions, among 
which the massing together of large classes of population in poorly ventilated 
and filthy quarters, as in military barracks for instance, was considered to 
be the most favorable predisposing cause. The disease is now perma- 
nently established in almost every large American city, though it has rarely 
assumed a severe epidemic form. In New York City, from 1866 to 1S72. the 
32 



498 THE NERVOUS DISEASES OF CHILDREN, 

annual deaths from this disease, according to Dr. Smith's statistics, varied 
from eighteen to forty-eight. A very severe epidemic occurred in December, 
1 87 1, and lasted well into the summer of 1872, so that 782 deaths, chiefly in 
children, resulted from cerebro-spinal fever in this city alone. Since that 
time the annual deaths have varied between 97, in 1878, and 461, in 188 1. 
The latest epidemic, though a small one, which has been most carefully ob- 
served by competent physicians, was the epidemic of Lonaconing, of which 
an account is given in the article by Flexner and Barker referred to above. 
This town is situated in the Alleghany Mountains and contains about five 
thousand inhabitants. A muddy stream which passes through the town, 
receiving most of the sewage, appears to have been responsible for the spread- 
ing of the epidemic. All the conditions, including the overcrowding of 
miners in filthy houses, were favorable to the spread of the disease. 

The disease is common both to man and beast. A serious epidemic 
occurred in New York City in 1871, and was at once recognized as a filth 
disease, for it first affected the horses in the large and overcrowded stables 
of the car and stage lines. A few individuals were soon similarly afflicted, 
but it is doubtful whether the disease was transmitted from the animals to 
the men who were in charge of them. The epidemic which occurred in 1872 
was evidently related to this same outbreak in animals in 1871. Though 
the disease is bred by filth, it may unquestionably also be carried, or at least 
transmitted, to persons, and particularly to children living in excellent hy- 
gienic surroundings, and many of us have seen such cases in the habitations 
of the richest as well as in the families of the working-classes. The disease 
may attack those in good health, but is even more apt to strike those whose 
health has been injured by previous disease or by fatigue. Dr. Smith quotes 
Frothingham as an authority for the statement that in a brigade of the Army 
of the Potomac which was attacked by this epidemic, the men were almost 
exhausted from excessive drilling. 

The disease shows no distinction between the sexes, at least in children. 
Dr. Smith reports 105 cases occurring in his practice, of which 59 were in males 
and 46 in females ; 91 of these cases being in children. While persons of 
every age may be attacked by the disease, it is unquestionably more liable to 
attack children in the earlier years of life than at any other period. It is in- 
teresting to note the statistics for a single year, 1883, as given by Smith for 
New York City : 

Under 1 year 57 1 From 1 5 to 20 years 15 

From 1 to 2 years 31 From 20 to 25 " 7 

From 25 to 30 " 3 

From 30 to 35 " 4 

From 35 to 40 " 3 

After this scattering cases. 



From 


2 to 


3 


From 


3 to 


4 


From 


4 to 


5 


From 


5 to 


10 


From 


10 to 


15 



22 

12 

9 

37 
18 



The youngest case which I have had opportunity to see was in a child, aged 
three months, who died within three days of the beginning of the disease. 



MENINGITIS. 499 

Symptoms. — The majority of the symptoms will natu- 
rally resemble those occurring in other forms of meningitis. 
The onset of the disease is characteristically sudden. A 
child in perfect health is suddenly seized with headache, 
vomiting, and either slight rigor or convulsions. In the 
milder cases a few days of malaise and of slight nausea may 
precede the onset of the other symptoms. The first severe 
symptoms are promptly followed by a stupor, which is apt 
soon to deepen into profound coma. I remember the case 
of a child, one year of age, in which a slight headache, 
vomiting, convulsions, rigidity of the neck, deep coma, and 
strabismus all developed within twenty-four hours, and 
death ensued within forty-eight. Severe neuralgic pains 
are frequent in the earlier stages of the disease. The pupils 
are unequal and dilated. The vomiting is of the cerebral 
order and occurs on the first or the second day in the 
vast majority of the cases. The fever is, as a rule, high, 
varying between 103 and 105 F. ; in one rapidly fatal case 
it reached 107 F. a few hours before death on the second 
day. 

The meningeal symptoms are developed in a very much 
shorter period of time than in other forms of meningitis. We 
do not have the slow progress from stupor to coma which 
we find in the tubercular type ; often the coma is deep 
from the very beginning. Delirium may alternate with in- 
tense coma. In some of the cases in which the coma is not 
profound, great restlessness takes the place of the stuporous 
condition. The child is, as a rule, extremely sensitive, even 
hyperaesthetic. The slightest touch of any part of the bodv, 
the mere weight of the bedclothes, is often sufficient to 
elicit shrill cries. This hyperassthesia is explained quite 
readily by the irritation of all the posterior root-fibres by 
the meningeal exudate. Contractions of the various mus- 
cles occur at an early stage of the disease. The head is 
firmly retracted, opisthotonos is distinctly developed, the 
thighs and legs are in a flexed position, and the arms and 
hands may be distinctly contractured. A hemiplegia or an 
alternate form of paralysis, or a monoplegia, together with 
cranial nerve palsies, may be made out in some patients. 
The deep reflexes are exaggerated, and the superficial 



500 THE NERVOUS DISEASES OF CHILDREN. 

reflexes are diminished. Convulsions occur more fre- 
quently in this form than in any other. 

Vasomotor disturbances in the skin merit special atten- 
tion, for they have given rise to some of the designations 
by which the disease is known. The " tache cerebrale " 
was formerly considered to be a rather important symptom 
of all forms of meningitis ; but it has lost its pathognomonic 
value, as it occurs in many other diseases. The skin pre- 
sents a peculiar mottling in the first or second week of the 
disease, and particularly when the temperature is low. 
Small red points and large bluish spots, due to exudation 
of blood under the surface, also appear, and were seen fre- 
quently enough to justify the term " spotted fever." But it 
is doubtful whether these spots deserved to be raised to the 
dignity of symptoms, and they are surely not an integral 
part of the disease, for in European epidemics these peculiar 
extravasations have not been regularly observed. Herpes 
occurs, and Smith refers to the occurrence of erysipelas ; 
but the latter is evidently entirely independent of the men- 
ingitis ; it is a complicating condition, and not in any sense 
a symptom of the epidemic form. 

The organs of special senses are frequently affected in epidemic menin- 
gitis, and possibly more frequently than in other forms. A hyperaemic and 
inflammatory condition of the entire eyeball is a common occurrence. The 
media may become cloudy and the various structures may become adherent 
to one another ; occasionally ulcerations of the cornea and perforation of the 
eye with total loss of vision may occur. According to Knapp, as quoted by 
Smith, the nature of the eye affection is a purulent choroiditis, probably met- 
astatic. In some cases a double optic neuritis occurs, and from this, as well 
as from the inflammatory conditions of the eyeball, total blindness may re- 
sult. Not a few of those who recover from cerebro-spinal meningitis are 
afflicted with permanent blindness as the result of this dreadful scourge. 

The hearing is often as seriously impaired as vision, and severe otitis 
media, ending in suppuration, with perforation of the drum and all its se- 
quences, is a common occurrence. In other instances loss of hearing is evi- 
dently due to more central causes, and is developed only after recovery from 
the main disease. It is unfortunate that the loss of hearing is apt to be bi- 
lateral and complete. According to Smith's statistics from the epidemic in 
1872, about one in every ten patients became deaf. But he states that in the 
milder form of cerebro-spinal meningitis which has prevailed since 1872 the 
proportionate number that has been thus affected has been less, and the 
same may be said with reference to loss of sight. Knapp reports that 



MENINGITIS. 5 1 

among twenty-nine cases of total deafness occurring after cerebro-spinal 
meningitis only one seemed to give evidence of hearing afterward. 

Morbid Anatomy and Pathology. — We need not 
again insist upon the microbic origin of the disease. If the 
exudate is examined the micrococcus lanceolatus or some 
other micro-organism can be found. The chief anatomical 
characteristics of the disease are an intense hypersemic con- 
dition of the meninges and of the brain, and this may be 
the sole morbid condition if the patient has died in the very 
early stage of the disease. If it has lasted more than a few 
days, pus is visible to the naked eye under the arachnoid. 
This membrane loses its transparency and begins to ap- 
pear cloudy, the cloudiness being most apparent along the 
course of the vessels from which the exudation undoubt- 
edly takes place. 

The pus is found both in the meshes of the pia, and un- 
der the pia between it and the cortex. The fibrinous puru- 
lent layer will be found adherent to the pia, and can usually 
be removed together with this membrane. These purulent 
layers can be drawn out of the fissures, leaving discolored 
tissue underneath. This purulent exudation covers not 
only the fissures of the convexity, but extends with equal 
frequency over the base of the brain, and in those very 
spaces in which we are accustomed to look for tubercular 
deposits. In addition to the exudation over the brain 
proper, an equally thick layer can often be found over the 
greater part of the spinal cord, holding the same relations to 
the spinal meninges that it does to the cerebral coverings. 
The blood is apt to be clotted in the large veins and sinuses, 
and such clots may be of a purulent character. The ventricu- 
lar fluid is, as a rule, increased, and in the more violent forms 
may contain small floccules of fibrin or fibrinous pus. 

Many changes occur in other organs of the body, but they are such as 
can be noted in almost every case of a person dying from an infectious dis- 
ease. Thus we may find hypostatic pneumonia, or varying degrees of 
bronchitis and atelectasis ; all the serous membranes may be in a condition 
of inflammation. The spleen is almost invariably enlarged, while the other 
abdominal viscera are found in a condition of decided hyperemia ; the kid- 
neys may be in a condition of acute congestion, and according to Welch an 
acute diffuse nephritis is occasionally present. 



502 THE NERVOUS DISEASES OF CHILDREN. 

Differential Diagnosis. — The only point to be con- 
sidered in this respect is the differentiation of this form 
from other forms of meningitis. This differentiation can 
be made only by reference to the course of the disease, 
which advances much more rapidly than in the tubercular 
or other types. The mental symptoms are, above all things, 
developed much more actively and vehemently, in keeping 
with the early exudation of pus over the surface of the 
brain. The temperature is higher in the earliest period of 
the disease. The known occurrence of similar disease in 
the neighborhood or in the same city, the condition of the 
environment of the patient, and the exclusion of those facts 
which tend to prove the presence of tubercular disease will 
help to make the diagnosis of the epidemic form much more 
certain. 

Prognosis. — The disease is unquestionably one of the 
most fatal diseases of childhood, and if it does not prove 
fatal the condition of the survivor is often so distressing 
that death would have been preferable. About one-half of 
the patients make a fair recovery. The duration of the 
coma is as reliable a sign as any in giving a prognosis. 
Cases in which the coma is rapidly developed and does not 
show any sign of receding within the first week or two are 
almost certain to end fatally. If the coma has been slow to 
develop, the inference is justified that the process is a less 
intense one, and there is in so far a hope of recovery. But 
even in these cases if the coma which has once been devel- 
oped remains stationary for a week or more, the chances of 
recovery lessen with almost every hour that the coma con- 
tinues. In spite of all rules that may be laid down children 
whom we have reason to expect to recover take a turn for 
the worse, and not a few of those who have been given 
up by the most careful and experienced physicians make 
good recoveries ; but in others, in whom the disease drags 
along, the final issue is simply deferred, and death may 
result from exhaustion, as in one of my own cases, as late 
as four months after the onset of the disease. 

Treatment. — The treatment of this special form of 
meningitis can differ in no respect from that noted in con- 
nection with the discussion of the tubercular and simple 



MENINGITIS. 503 

forms of meningitis. In view of our present knowledge of 
the causation of the disease patients should be placed under 
favorable hygienic conditions. The greatest care should be 
exercised in feeding the child, in stimulating cardiac action, 
and in keeping the little one absolutely quiet. Although 
the disease has not been proved to be contagious, the pa- 
tient should be strictly isolated and care should be taken 
not to expose those who are especially predisposed to the 
disease. 

MENINGITIS DUE TO OTHER CAUSES.* 

In the preceding pages we have considered the most frequent types of 
meningitis in children. There are a few other forms to which we must 
allude briefly, although reference has been made to some of them in the be- 
ginning of this chapter. 

I. Meningitis due to traumatism has been mentioned in connection with 
acute meningitis ; it is much rarer in children than in adults, for reasons 
which it is hardly necessary to explain ; though the injuries which children re- 
ceive may be relatively slight, meningeal disturbances follow upon them in 
some instances. A fall from a chair, a fall down the stairs, or a blow dealt 
upon the head of the child, to which little heed is paid at the time, may be 
the starting-point of the meningitis. At times the same slight traumatic fac- 
tor is the cause of an epilepsy developing after six months, or even after a 
year, or still later (see chapter on Epilepsy). In these children a local menin- 
gitis or meningo-encephalitis may be considered to be the actual lesion re- 
sponsible for the epilepsy. With such cases we have no concern at present. 
We must take into account, however, those patients in whom some trauma- 
tism to the skull is followed by the symptoms characteristic of meningitis. 
The inflammatory process may involve both the dura and the pia, and may 
be either serous or purulent. 

In these days of cranial surgery, another form of (purulent) meningitis is 
to be observed in children and in adults after operative procedures for the re- 
lief of epilepsy or other brain diseases. The aseptic principles of modern sur- 
gery are calculated to prevent such complications. It is exceptional to see 
this condition in cases in which a simple trephining operation has been done, 
but if the dura has been opened the danger of meningitis from septic infection 
is very much greater, and should be guarded against. Nor does the danger 
end with the operation. A lad of fourteen, who had been operated on for 
epilepsy, had done extremely well after the operation. On the twelfth day a 
violent purulent meningitis set in which proved fatal within a week. Be- 
tween the eighth and the tenth day the dressings had been changed for the 
first time (not by the surgeon in charge), and in some inexplicable way the 
wound had become infected at that time. The autopsy showed a purulent 

* For " Meningitis serosa " see page 514. 



504 THE NERVOUS DISEASES OF CHILDREN. 

local encephalitis, with a meningitis spread over the entire convexity of both 
hemispheres as well as at the base, evidently emanating from the site of the 
operation. 



The prognosis of a meningitis developing after opera- 
tion is, on the whole, excessively grave. The only advice 
to be given is that as soon as the temperature rises, and as 
soon as the first symptoms of a suppurative meningitis set 
in, the wound should be opened at once, and an effort made 
to treat the condition according to the best surgical prin- 
ciples. The same applies to those cases of meningitis which 
follow upon traumatic injuries, if the meningeal symptoms 
can be clearly traced to the preceding injury. A careful 
examination of the skull should be made in order to deter- 
mine, if possible, where the injury has been inflicted. If 
the slightest depression can be felt trephining should be 
done over this region, and if no changes in the skull can be 
made out, but the symptoms point to an incipient meningitis, 
any abrasion of the scalp, or any extravasation of blood un- 
der the scalp, should be a sufficient guide as to the site of 
the operation. I am firmly convinced that much good can 
be done by proper and timely surgical interference in these 
cases, and little harm will result even if the operation should 
prove that no tangible local injury has been done to the 
brain or skull. To be sure the operation should not be un- 
dertaken if the child's general condition is such that it will 
not bear the shock of the operation. In such circumstances 
the calm judgment of an experienced surgeon or physician 
will be of great value, but it should be distinctly stated that 
coma, however profound, or recurrent convulsions, do not 
constitute a contra-indication to operative interference. 

II. Meningitis in a purulent form may result from dis- 
ease of the ear. The aurists are well aware of the danger 
lurking in every form of disease of the mastoid and of the 
inner or middle ear; for if the pus that is formed in either of 
these regions is not discharged outward, the danger of its 
causing a purulent meningitis by a direct discharge inward, 
or through caries of the petrous bone, is much to be feared. 
Chronic ear trouble, so slight that little attention is paid 
to it, may persist for years before causing a purulent men- 



MENINGITIS. 505 

ingitis.* Since aural surgeons have become accustomed 
to operate promptly upon the appearance of symptoms 
pointing to suppuration in the mastoid process, or in the 
other bony parts of the ear, these forms of purulent menin- 
gitis from ear disease are far less frequent than they were 
formerly. The symptoms which point to an incipient 
meningitis can be distinguished in many cases, though not 
in all, from those due to the presence of pus within the ear 
structures alone. In addition to the intense pain, the pres- 
ence of pus in the ear may cause giddiness, intense vertigo, 
vomiting, and even slight stupor, but if the headaches be- 
come general and most intense, if the child becomes coma- 
tose, if the pulse is either slowed up or very much acceler- 
ated, if the neck becomes sensitive and rigid, and if a slight 
optic neuritis should set in in one or both eyes, it is certain 
that the pus has passed beyond the limits of the ear and has 
set up a meningitis, or possibly an abscess. Under such 
conditions it is imperative to operate, giving the pus a 
chance to discharge outward, and to lay bare, if necessary, 
the parts of the brain which are most apt to be involved in 
ear disease. It will be advisable at the start thoroughly to 
explore the mastoid, as well as the middle and inner ear, be- 
fore attempting to trephine over the temporal convolutions, 
but if the operations upon the bony parts of the ear do not 
give prompt relief the cranial operation should be done. The 
aural surgeons are opposed to operations in cases of purulent 
meningitis, but as long as the meningitis is strictly localized 
and confined to the parts in immediate juxtaposition to the 
ear, there is every reason to advise an exploratory operation 
to secure an exit for the pus, which, if confined to these 
parts, is bound to cause a general suppurative meningitis. 

If suppurative meningitis is due to disease of the mas- 
toid, the pus is more likely to find its way either to the cere- 
bellum or to the base of the brain. In diseases of the middle 
and inner ear, the meningitis is more apt to be developed 



* The author saw this well illustrated in a lad of fourteen (a patient of Dr. Wiener). 
who since his fourth year had* had chronic ear disease ; without special cause this old 
trouble was lighted up and ended in death within one week. The autopsy disclosed a 
wide-spread purulent meningitis from caries of the right petrous bone. The morbid 
condition was most marked over the left temporo-sphenoidal lobe. 



$06 THE NERVOUS DISEASES OF CHILDREN. 

in the regions over the first and second temporal convolu- 
tions. In endeavoring to locate the proper region for open- 
ing the skull in cases of ear disease it is best to select a point 
that is reached by going one and a quarter inch back of 
the external auditory meatus, and from this point one and a 
quarter inch upward. If the surgeon remembers that a 
line drawn from the outer angle of the orbit horizontally 
across the skull gives the approximate position of the fis- 
sure of Sylvius, and therefore the upper limits of the tem- 
poral convolutions, he cannot well fail to open the skull over 
the part of the brain that comes into question in these cases.* 

Meningitis may also be caused by a tumor or an abscess 
in adjacent parts of the brain ; but in such cases the menin- 
gitis is comparatively of little importance, and if it gives 
rise to distinct symptoms simply helps to intensify those 
caused by the chief morbid process. Purulent meningitis is 
sometimes due to disease of the nose, to erysipelas, and to 
purulent disease of the eye. If so, the antecedent condi- 
tions will leave little doubt of the cause of the meningitis, 
and the subsequent symptoms will not vary from the men- 
ingitis following upon other causes. 

III. Many of the infectious diseases of children lead to 
meningitis. Among these are measles, scarlatina, small-pox, 
and even rheumatism and influenza, but, above all, typhoid 
fever and pneumonia. 

Meningitis has been found present in the autopsies upon 
persons dead from typhoid fever, but is by no means pres- 
ent in all those forms of typhoid fever in which at the begin- 
ning or in the later stage of the disease delirium and coma 
are developed in connection with the fever. If a child have 
typhoid, the diagnosis of additional meningitis should not 
be made unless there are very positive symptoms, such as 
decided rigidity of the neck, hyperesthesia of part or of the 
entire body, and cranial-nerve palsies. 

Brain symptoms are not infrequent complications in 
acute pneumonia, and much attention has been directed to 
these cerebral complications by Holt and others ; but menin- 
geal disease unquestionably occurs in a number of instances, 
and we can the more readily understand the connection 

* See chapter on " Abscess." 



MENINGITIS. 507 

between the two diseases since Fraenkel's diplococcus has 
been found in the inflamed lung, and in the meningeal exu- 
date. The same coccus is also found in the epidemic puru- 
lent form of meningitis. 

IV. Among the many curious and surprising sequelae 
of influenza which have been described, none has been more 
striking than the occasional occurrence of cerebral and cere- 
brospinal meningitis during or immediately after the acute 
stage of the disease. For the present the evidence is in favor 
of an encephalitis, rather than a meningitis, as the cause of 
this spinal form of disease. (See p. 508.) 

Inflammation of the meninges also follows upon general 
septicasmic processes, but this special causation is much 
more common in the adult than in the child. The typical 
septicaemic meningitis is that form which we encounter in 
connection with the puerperal state in women, and from 
which I have seen one most remarkable recovery. There 
is no form of septicaemic meningitis in children quite as 
typical as this puerperal meningitis, unless it be that which 
occasionally occurs with septic bone disease or with ulcer- 
ative endocarditis. All these cases are no doubt due to 
definite micro-organisms which are carried from the focus 
of disease to the brain and its coverings. In ulcerative en- 
docarditis, embolism of the smaller vessels by the accumu- 
lation of micrococci, as we know happens in other organs, 
is a distinct possibility. 

Specific disease, which is a very frequent cause of acute 
and chronic meningitis in the adult, does not play an impor- 
tant role in the meningitis of early life. I have not seen a 
single instance of acquired or hereditary syphilis in a child 
in which the diagnosis of a specific meningitis seemed to be 
the most probable one. 

All the forms of meningitis hitherto described belong 
to the category of acute meningitis. Subacute or chronic 
forms are extremely rare in children, for the very reason 
that the factors which lead to chronic forms of meningitis 
in the adult are not operative in early life. 

Syphilis does not exert its powerful influence, and 
chronic alcoholism and metallic poisoning are, fortunately, 
comparatively rare in earlier years. 



508 THE NERVOUS DISEASES OF CHILDREN. 



ACUTE ENCEPHALITIS. 

We cannot close the discussion of meningitis without reference to acute 
encephalitis, which is frequently associated with inflammation of the pia, but 
is also at times developed independently of any meningeal trouble. In men- 
ingitis due to traumatic injury, or to acute and chronic intoxication, some 
encephalitis occurs by simple extension of the inflammation. During the past 
few years, Strumpell, Fiirbringer, and others have endeavored to establish 
a form of acute hemorrhagic encephalitis which is developed commonly after 
some acute infectious disease, particularly after influenza. 

Simple acute encephalitis can be recognized by the following signs : 
The disease is very apt to attack children before the age of puberty. For a 
few days previous to the full development of the disease the child complains 
of headache, of dizziness, and is irritable or depressed ; drowsiness, gradually 
developing into coma, soon sets in. Rigors and slight elevations of temper- 
ature point to the development of an acute infectious trouble. The loss of 
consciousness need not be complete, and distinct remissions occur during the 
first week of the disease. Hours of stupor are followed by a condition of 
wakefulness and restlessness. The pupillary reflexes may remain normal or 
may be sluggish ; the deep and superficial reflexes are not altered. A rigid- 
ity of the neck and slight opisthotonos are early symptoms. Paralysis, in 
the form of monoplegia or a hemiplegia, is observed at an early day, and may 
or may not be associated with aphasia. Ocular and other cranial nerve pal- 
sies are developed, and if they set in in an acute fashion, the condition may 
remind one very easily of a basilar meningitis, with the exception of the in- 
complete loss of consciousness. Respiration may become irregular, and the 
cardiac action is either accelerated, irregular, or diminished. Optic neuritis 
has been observed in several such cases. The symptoms will vary very much 
according to the seat of the disease. Loss of consciousness, convulsions, and 
palsies will be more frequent in encephalitis of the convexity, whereas cranial 
nerve palsies, dizziness, difficulties in deglutition and in articulation may be 
expected if the encephalitis is developed chiefly in the basilar structures. 

The course of the disease will vary also according to the intensity of the 
encephalitis. In some of the cases the coma deepens, the patient may linger 
on for two or three weeks, but finally succumbs to the disease, the entire 
course reminding one very much of the pyasmic forms of encephalitis. In 
other patients, after the disease has lasted for a week or two, prolonged 
remissions and complete recoveries may set in. 

As an instance of encephalitis following influenza, I wish to refer to the 
following case, although the possibility of a meningitis being associated with 
the encephalitis cannot be denied. The patient and several other members 
of the family had passed through the usual attacks of influenza four days 
previous to my first visit. The girl, eighteen years of age, had been badly 
frightened by the falling of a picture, which was, however, a mere incident 
and not an etiological factor in the case. That same evening she began to 
vomit and complained of severe headaches. The family physician, who ex- 



MENINGITIS. 509 

amined her, found a temperature of 103 F., and noticed at once a slight re- 
traction of the neck. For the next few days the temperature varied between 
101 and 103 F. The rigidity of the neck became more pronounced, but 
the pulse and respiration were not sensibly affected at any time, the former 
being at the rate of 90 and the respirations varying between 16 and 20 a 
minute. On the fourth day of the disease there was a very marked rigidity of 
the neck and great painfulness over the cervical and dorsal spine ; excessive 
muscular tenderness in the lower extremities, such as is frequently found in 
cases of simple influenza. The deep reflexes were lively, but not excessively 
exaggerated. The patient was drowsy ; if left to herself would sleep, but 
when aroused answered all questions intelligently. She presented in addition 
a marked paralysis of the rectus externus in both eyes. There was loss of light 
reflexes and sluggishness of contraction during accommodation in both eyes, 
but there were no other third-nerve symptoms. The papillae were somewhat 
swollen. These symptoms continued, with slight changes, for two weeks ; 
then she began to improve slowly ; four weeks after the onset of the first 
symptoms the patient was entirely well. 

These cases surely bear a strong resemblance to the ordinary forms of 
meningitis, and were it not for the evidence furnished by Fiirbringer and 
Koenigsdorf that encephalitis is the actual condition, the diagnosis of a 
meningitis could be defended quite as readily. 

Morbid Anatomy. — As far as can be determined at the present time, 
the morbid process is a simple hemorrhagic encephalitis. The inflammatory 
areas are small and strictly circumscribed, and may be developed in symmet- 
rical parts of the brain. On superficial examination, the brain tissue appears 
hyperasmic and dotted with small red points ; the areas of inflammation are a 
little softer to the touch than normal tissue. On microscopical examination 
the smaller vessels are found dilated to their utmost capacity, filled with 
blood, some of which has evidently been exuded into the neighboring tissue. 
Leucocytes, granular cells, and a proliferation of the cells of the neuroglia 
complete the microscopical picture. There may be slight destruction of the 
nervous elements, but this will depend upon the intensity of the process. 
The entire encephalitis is, no doubt, of microbic origin, but whether the small 
areas of inflammation are the direct result of the accumulation of microbes 
has not been determined, 

The prognosis will naturally vary according to the intensity of the symp- 
toms. A fatal issue is apt to follow in those cases in which the symptoms, 
coming on with great violence, point to an intensely septic process. If the 
condition of coma is reached by slow stages and remissions are observed 
within the first week or ten days, a favorable turn may be expected. 

The treatment will consist simply in absolute quiet, the application of cold 
to the head and nape of the neck, and in the administration of purgatives 
(calomel is by far the best). 

Polio-encephalitis Superior. — A disease due to the inflammation 
of the gray matter of the floor of the fourth ventricle and of the aqueduct of 
Sylvius, had been described by Wernicke, Thomsen, and others. This dis- 
ease has bee-n observed in the adult, and is remarkably frequent in persons 



5io 



THE NERVOUS DISEASES OF CHILDREN. 



presenting the symptoms of chronic alcoholism. This special form would 
naturally be a rarity in children ; the symptoms are, however, not unlike that 
form of encephalitis which has been described in connection with influenza. 
It is of particular interest, also, because a similar condition is at times asso- 
ciated with polio- myelitis. We have often referred to the close relation be- 
tween the gray matter in the spinal cord and the gray matter which harbors 
the nuclei of the cranial nerves. We can readily understand why the same 
morbid process should at times affect the cerebral, and at other times the 
spinal portion of this central gray matter. According to the distribution of 




Fig. 136— Case of Unilateral Nuclear Palsy. (Wiener.) Hypoglossal nucleus, left 
side. Photomicrograph from a section stained after Pal, showing some degenerated 
ganglion cells. 

the inflammation in the region of the ocular nerve nuclei, or in the vicinity of 
the nuclei of the tenth and twelfth nerves, we distinguish between polio- 
encephalitis superior and inferior. The disease in which ocular nerve symp- 
toms (ophthalmoplegia, partial or complete) have been associated with polio- 
myelitis, is termed polio-encephalomyelitis. This form has been described 
by Rosenthal, Seeligmiiller, Guinon, myself, and others. But these diseases 
are, on the whole, extremely rare, and do not, as a rule, begin until childhood 
is past. The cases also take a more or less subacute course. 

Bulbar Palsies.* — Diseases of the pons and medulla are very rare in 
children ; but the above discussion of nuclear diseases leads to the mention 



* The author had some hesitation in discussing these diseases in this chapter ; but 
they are too rare to warrant discussion in a separate chapter, and it did not seem 
desirable to add them to the sections on congenital nuclear palsies. 



MENINGITIS. 5 1 1 

of progressive bulbar palsy. This disease in the adult bears a close clinical 
and anatomical relation to progressive amyotrophy and to amyotrophic lateral 
sclerosis. With the chronic diseases of adults we are not concerned, but a 
short allusion may be made to progressive bulbar palsy of earlier years, 
which is a rare disease, but, like the adult form, is due to a primary degen- 
eration of the nuclei of the lower cranial nerves.* A case of this descrip- 
tion, from my clinic, was carefully studied and described by Dr. Wiener. 

The symptoms of unilateral bulbar palsy were discovered quite acciden- 
tally in a young man who had been under treatment by his physician for tu- 
bercular glands and pharyngitis. He presented : i . A very marked deviation 
of the tongue to the right when protruded. 2. Distinct atrophy of the me- 
dian portion of the right half of the tongue. 3. The faradaic response of 



;<•: 




Fig. 137. — Same Case as Fig. 136. Hypoglossal nucleus, right side, showing absence 
and extreme degeneration of ganglion cells. 

the right half of tongue was much diminished ; the contractions were slug- 
gish, there was increased galvanic excitability of the right side (K. C. C.< 
A. C. C.) ; the contractions were slow and wave-like. 4. Taste and tactile sen- 
sations were normal. 5. Deviation of the soft palate and of the uvula to the 
left side. On phonation the paralysis of the right side became more marked. 
6. Great difficulty in deglutition. 7. Dysphonia due to disease of the right 
recurrent laryngeal nerve. 

Other cranial nerve functions were normal. All reflexes normal. 

A gradual progression of all the symptoms led to a fatal issue from respira- 
tory failure. Examination of the brain revealed a distinct degeneration of the 
hypoglossal and vago-accessorius nuclei of the right side, with degeneration 
of the respiratory column. In the hypoglossal nucleus, the ganglion cells 
were greatly changed (atrophied, shrunken, and granular). The ground 

* No special reference is here made to the acute and the pseudo-bulbar palsies, as 
both these conditions are very rare in children. An acute polioencephalitis inferior in 
young subjects, giving rise to bulbar symptoms, has been described. 



512 THE NERVOUS DISEASES OE CHILDREN. 

substance did not stain as readily in the right as in the left, and was less 
compact ; the left hypoglossal nucleus was also slightly diseased (lower and 
outer portions). (See Figs. 136, 137.) 

The symptoms of bulbar palsy (paralysis and atrophy of the muscles of 
the palate, the tongue, the pharynx, and the larynx), were distinctly present. 
In some subjects there is additional paralysis of the lips and of the muscles 
of mastication, and also of the upper division of the facial distribution. 
The difficulties of deglutition and the dysarthria were characteristic ; all these 
symptoms justify the clinical designation of glosso-labio-pharyngeal paral- 
ysis. The unilateral character of the symptoms is worthy of note, but does 
not remove the case here recorded from the category of subacute or chronic 
bulbar palsies. 

The disease which involves the nuclei of the motor cranial nerves is occa- 
sionally associated with spastic symptoms, with increase of the deep reflexes, 
and with atrophy in the upper extremities. The clinical symptoms thus bear 
the closest resemblance to those of amyotrophic lateral sclerosis,* and the 
latter disease may well represent an extension of the bulbar process into the 
spinal cord ; the spinal symptoms of amyotrophic lateral sclerosis may pre- 
cede the bulbar symptoms for a long period of time. All these forms repre- 
sent a disease of both divisions of the motor tract. 

C. H. Brown has described an interesting case of amyotrophic lateral 
sclerosis with bulbar symptoms which exhibited the first signs at the age of 
twelve years or earlier. All the facial muscles were involved ; the boy could 
not laugh or swallow easily ; speech was nasal ; there was atrophy of the 
sterno-cleido-mastoid of the left side, and to a lesser extent of the right. 
Other neck muscles weak. Considerable atrophy around the shoulder gir- 
dle, of all of the arm muscles, and of the interossei and the thenar group, 
fibrillary twitchings and increased reflexes completed the series of symptoms. 
Progressive bulbar paralysis in children has also been described by Hoff- 
man, Remak, and Londe. Eisenlohr has described a bulbar form of infantile 
spinal paralysis. 

BIBLIOGRAPHY. 

MENINGITIS (SIMPLE AND TUBERCULAR). 

Freyhan: Deutsche Med. Wochenschr., 1894, p. 709. 
Henoch : Vorlesungen, etc., seventh edition, p. 297. 
Hirschherg: Bull. General de Therapeutique, 1894. 
Jacobi : Keating's Cyclopaedia (Tubercular Meningitis). 
Minot : Pepper's System of Medicine, vol. v., p. 723. 
Ord and Waterhouse : Lancet, March 10, 1894. j 

Pollitzer: Jahrb. f. Kinderheilkunde, 1863, Bd. VI., p. 40. 
(See also the text-books of Eustace Smith, Henoch, Rilliet et Barthez, and 
Donkin.) 

* In children a post-diphtheritic palsy may for a time resemble a bulbar palsy. 



MENINGITIS. 5 1 3 



EPIDEMIC CEREBRO-SPINAL MENINGITIS. 

Eberth : Deutsch. Arch. f. kl. Med. 

Flexner and Barker : American Journal of the Medical Sciences, February, 

1894 (ample literature). 
Kahler : Prager Med. Wochenschr., 1887. 
Leyden: Centralbl. f. klin. Med., 1883. 
Neuman and Schafer : Virchow's Arch., 1887, Bd. CIX. 
Roux: Lyon Medical, 1889. 

Smith, J. Lewis : Text-Book, seventh edition, 1890, p. 470. 
Striimpell : Deutsch. Arch. f. kl. Med., Bd. XXX. 
Weichselbaum : Fortschritte der Med., 1887. 



ACUTE ENCEPHALITIS. 

Friedmann : Arch. f. Psychiatrie, Bd. XXL, p. 461 et seq. 
Fiirbringer : Deutsche Med. Wochenschr., 1892, No. 3. 
Hayem : Etudes sur les Diverses formes d'Encephalite. Paris, 1868. 
Hirt : Pathologie u. Therapie, second edition, p. 223. 
Koenigsdorf : Deutsche Med. Wochenschr., 1892, No. 9. 
Leichtenstein : Deutsche Med. Wochenschr., 1890, No. 23. 
Putnam : Boston Medical and Surgical Journal, October 13, 1892. 
Striimpell : Arch. f. kl. Medicin, Bd. XLVIL, p. 53. 
Virchow: Virchow's Archiv, Bd. XXXVIII. , 44. 



BULBAR PALSIES. 

Brown : Journal of Nervous and Mental Disease, 1894, p. 707. 

Duchenne : Electrisation localisee, third edition, p. 570, 1872. 

Eisenlohr: Zeitschrift f. kl. Medicin, 1880. 

Eulenburg: Article Bulbar Paralyse in Eulenburg's Real-Encyclopaedie, 

third edition, vol. iv., 1894. 
Hoffman : Zeitschrift fur Nervenheilkunde, vol. i., p. 169. 
Londe : Revue de Medecine, 1893. 

Remak : Archiv f. Psychiatrie, vol. xxiii., p. 919 (ample literature). 
Wiener: New York Medical Journal, July 14, 1894 (contains literature of 

unilateral paisies). 



33 



CHAPTER XXVII. 

HYDROCEPHALUS. 

By hydrocephalus Ave mean an excessive accumulation 
of serous fluid within the cranium, either in the subdural 
spaces or in the ventricles. The former is termed exter- 
nal hydrocephalus ; the latter, internal hydrocephalus. 
It is convenient also to divide the cases into acute and 
chronic forms, and to subdivide the latter into congenital 
and acquired hydrocephalus. As the accumulation of fluid 
often follows upon other diseases, we distinguish a form of 
secondary hydrocephalus ; the term primary hydrocephalus 
remains restricted to the few cases in which the accumu- 
lation of fluid appears to be the sole morbid condition. 
Chronic hydrocephalus is by far the more important, for 
what might be said of truly acute hydrocephalus has already 
been said in connection with the primary forms of meningi- 
tis ; but a few remarks will be in order, at this juncture, in 
relation to the subject of acute hydrocephalus, neglecting 
for a time the hydrocephalic condition which accompanies 
every form of meningitis, and particularly the tubercular 
variety. 

Acute Hydrocephalus.— Quincke, in an able article 
on meningitis serosa states, that the term acute hydrocepha- 
lus should be made to cover the condition in which there is 
a purely idiopathic serous meningeal inflammation. In the 
cases which he describes, and of the existence of which there 
is no doubt, the symptoms point to a very constant increase 
of intracranial fluid in the subpial spaces and within the 
ventricles. Acute hydrocephalus is characterized generally 
by very sudden onset ; occasionally the symptoms come 
on in insidious fashion. Fever may be entirely wanting, or, 
if present, rarely exceeds 103 F. ; headache, rigidity of the 



HYDR O CEPHA LUS. 5 I 5 

neck, nausea, vomiting, stupor, coma, delirium, are the symp- 
toms present in this disease, and they are the same which 
we have insisted upon so frequently in connection with the 
various forms of meningitis. The pupils react sluggishly, 
and are often unequal ; optic neuritis is present ; convul- 
sions may occur, but are rare, and palsies of a cerebral 
order may be present, but are rarely either severe or lasting. 
Paralysis of the external rectus muscle is as frequent as any, 
and this can readily be explained by increased intracranial 
pressure. In milder cases the symptoms soon recede, and 
absolute recovery may take place within a few weeks. In 
the more serious forms the symptoms may closely resemble 
those of brain tumor. The disturbance of function due to 
increased intracranial pressure may bring about a fatal issue. 
Some of these types of meningitis serosa are no doubt idio- 
pathic, others may represent an acute exacerbation of a 
chronic hydrocephalus. These diseases occur rarely before 
the first year of life, but are most frequent between the age of 
one and five years. They may occur up to the age of thirty 
years, and even later. 

Meningitis serosa may be due to traumatic injury and 
to acute febrile disease, such as typhoid and pneumonia ; in 
the adult the excessive use of alcohol, and the pregnant 
state may be responsible for the development of the disease. 
It is probable that this serous form of meningitis is not of 
microbic origin. A distinction should be made in fact be- 
tween these cases and those in which some micrococci are 
known to be the definite cause. 

Pathology. — Acute hydrocephalus may be due to a 
number of different causes. First, to venous stasis, such as 
occurs in connection with severe heart disease. Secondly, 
venous stasis in the larger veins at the base, due to neoplasm 
in the posterior cranial fossa, may also be the direct cause oi 
acute dropsy of the brain. Thirdly, stasis in the lymphatic 
vessels, most frequently due to tumors in the posterior fossa, 
causing compression of the subarachnoid lymph spaces and 
of the aqueduct of Sylvius, are a sufficient cause of acute 
hydrocephalus. Fourth, any acute inflammatory process 
of the brain or of the meninges may lead to this form oi 
meningitis. The exact symptomatology of an affection of 



$l6 THE NERVOUS DISEASES OF CHILDREN. 

this nature occurring in a young person may be illustrated 
by the following history taken from Quincke : 

A boy, nine years old, who was said always to have had a large cranium, 
was struck upon the back of the head. He was unconscious for a few min- 
utes, and since the accident complained of pains in the occiput, which were 
generally slight, but occasionally much increased. With increasing headaches 
the boy grew pale, vomited, showed slight rigidity of the neck, and retraction 
of the head. The horizontal circumference of the head six months after the 
accident was 54^ ctm. No pain on percussion of the head, but pressure upon 
the upper cervical spinous processes was painful. Vision much diminished. 
The right eye had fair perception of light ; with the left the boy was able to 
count fingers. The pupils moderately dilated, reacting sluggishly; both 
papillae were atrophic. All movements were perfect. Serous fluid was with- 
drawn in considerable quantity on two different occasions by Quincke through 
a puncture in the lumbar region. Upon this there was improvement in the 
headaches, but sight remained permanently impaired. 

Chronic Hydrocephalus. — From this category of 
cases we may at once exclude those in which the increase of 
intracranial fluid is of a simple compensatory nature. Such 
an increase is found in many instances of congenital imper- 
fect development of the brain. A skull of average dimen- 
sions sometimes harbors a very small brain, the cranial cav- 
ity being filled by an increased amount of fluid. The fluid 
causes a bulging of all the cranial bones. The accumulation 
of fluid during the intra-uterine period may increase the size 
of the head to such an extent as to make it a serious obstacle 
to normal delivery. 

Deficient brain development is at times limited to one- 
half of the brain. An entire hemisphere may be trans- 
formed into a large serous sac, while the other half presents 
tolerably normal appearance. An entire hemisphere may 
consist of nothing more than a superficial layer of gray 
matter bordering upon a huge cyst which communicates 
with the ventricles. I have had opportunity to be present 
at an operation upon such a brain in which a cyst was di- 
agnosticated, but the size of the cyst not suspected. On 
opening this cyst the surgeon's probe could be passed many 
inches forward and backward, showing that the greater 
part of the hemisphere consisted of this cystic mass. The 
boy died about two weeks after the operation, when it was 



HYDR O CEP HA LUS. 5 1 7 

found that the entire hemisphere consisted of nothing else 
than a small layer of gray matter bordering a huge cystic 
cavity. But all these forms, however interesting they may 
be, have comparatively little value, and are far less frequent 
than those in which there is a condition of congenital hy- 
drocephalus. 

Congenital Hydrocephalus. — For reasons which are 
not well known an excessive accumulation of fluid may occur 
within the ventricular cavities during the intra-uterine pe- 
riod. The condition is found in children of absolutely 
healthy parents after normal pregnancies, and in families in 
which all other children have been entirely healthy. In 
some families, however, several children are born afflicted 
with this same trouble ; one or the other may survive while 
the majority are either still-born or die very soon after 
birth. Syphilis of the parents, alcoholism, tuberculosis in 
the father or mother, emotional excitement of the mother 
during pregnancy, injuries to the mother during this same 
state, all these factors have been held responsible for the 
condition, and no doubt are operative in some of these 
patients, while in the majority the actual cause remains un- 
known. If hydrocephalus is present at the time of birth 
the amount of fluid present may go on increasing within 
the first few days, or as long as the child lives. 

In this connection the observations made upon an infant whom I had 
occasion to examine in 1893 may be of some interest. When I saw it 
for the first time it was four weeks old. The mother had given birth to one 
other girl, three years previously, that had remained healthy and showed no 
signs of internal hydrocephalus. The father was a man of remarkably vig- 
orous health. During this second pregnancy the mother menstruated regu- 
larly. On account of the size of the head labor was extremely difficult, but 
the child was uninjured when born. The measurements which were taken 
at three different occasions will show the rapid increase of the fluid, as indi- 
cated by the rapid increase in the head measurements : 

Aug. 6. Aus. 8. Aug. 10. 
Inches. Inches. Inches. 

Horizontal circumference 20^ 21 1 ' 2 22 

Naso-occipital measurement 13^2 i- l z i-l-'i 

Bin-auricular measurement 12^ 13 * 2 13 

On the 9th, between the second and third measurements, a large amount 
of fluid was withdrawn by tapping the fontanelles. but it will be noticed that 



5 18 THE NERVOUS DISEASES OF CHILDREN. 

the fluid was rapidly replaced. The child lingered on for another week and 
then died, after having become greatly emaciated. More than a week before 
its death it could not be fed by the mouth or per rectum. 

But all cases of congenital hydrocephalus do not take 
such a serious turn, and in some of them the accumulation 
of fluid ceases and a tolerably fair and even normal mental 
development may ensue. The protruding occipital bones 
clearly visible on so many bald heads point to a moderate 
amount of internal hydrocephalus in the earlier years of 
life. Some of these may have been acquired in the earlier 
period of life and are not necessarily congenital in origin. 
The fluid accumulates most readily within the lateral ven- 
tricles, both in the anterior and in the posterior horns. The 
aqueduct of Sylvius may be distended into a funnel shape 
by the increased fluid, but the fourth ventricle, as a rule, 
suffers very little. The brain is compressed by the ac- 
cumulation of fluid, and is often so flattened and thinned 
that its thickness is not greater than that of ordinary thick 
paper. The white matter seems to yield more readily than 
the gray, and I have been struck, in a number of different 
brains, by the fact that the function of the parts remained 
normal in spite of the extreme thinness of the cerebral tissue. 
Thus in one instance in which both occipital lobes were re- 
duced to the thickness of paper, sight had remained entirely 
normal up to the age of two years, at which time the patient 
died. The membranes are, as a rule, normal, or else show 
only very slight thinning, and on microscopical examination 
only the slightest traces of acute inflammation or none at 
all. The choroid plexuses are generally thickened, as is 
also the limiting membrane of the ventricles. 

The fontanelles bulge and pulsate distinctly. The con- 
figuration of the skull is modified by the excess of internal 
hydrocephalic fluid. If the hydrocephalus is congenital, or 
if it has occurred in very early life, the bones yield to the 
increased pressure within and the skull becomes distended 
in all directions. In keeping with the greater accumulation 
of fluid in the anterior and posterior horns of the ventricles 
and the protrusion of corresponding parts of the brain, the 
greatest amount of bulging is observed in the frontal and oc- 
cipital bones respectively. The effect of the excessive ac- 



HYDROCEPHAL US. 5 1 9 

cumulation of fluid in the posterior horns of the ventricles 
results in a great bulging of the occipital bones, with marked 
increase in the transverse occipital diameter of the skull. 
If the child survives, the congenital internal hydrocephalus 
gives rise to a number of symptoms pointing to deficient 
cerebral development. On account of the size and weight 
of the head the child is rarely able to carry the head unsup- 
ported. As it grows older it may be able to sit in a chair, 
but the head generally inclines to one side or the other, or 
forward, in which case it may rest upon the chest. Defec- 
tive mental development is present in the majority of these 
cases, ranging between total idiocy and varying degrees of 
imbecility ; some of the children acquiring a slight use of 
language and others learning to utter only a few words. 
Cerebral palsies with typical spastic contractures are quite 
frequent, and convulsive seizures amounting to a chronic 
epilepsy often add to the burdens of the child, and to the 
distress of the mother who is to care for it. In some cases 
there is total blindness, due to optic-nerve compression, but 
the most surprising feature of all is the remarkable pres- 
ervation of some of the cerebral functions in spite of the 
great disadvantages under which the brain labors. 

Acquired Internal Hydrocephalus. — Chronic internal hydro- 
cephalus, not of congenital origin, is a rare disease, and it is at least in rare 
instances only that the diagnosis of such a form of internal hydrocephalus 
can be made, and if made, the cause which gives rise to it is so far more im- 
portant that the internal hydrocephalus is a mere incident and does not de- 
serve much notice. This acquired internal hydrocephalus may be of pri- 
mary origin or it may be due to some mechanical obstruction causing venous 
stasis. The primary form may be a symptom of severe general anaemia, and 
under such conditions it is a counterpart of serous transudation occurring in 
various cavities of the body. It may also occur in children who have rick- 
ets, or in those whose skull is sufficiently yielding to permit enlargement of 
the cranial cavity. The increase of fluid is to a certain extent compensatory 
in such cases. 

Internal hydrocephalus due to mechanical obstruction of the veins of 
Galen, was first described by Whytt. The commonest form of obstruction 
is that due to tumor in the posterior fossa. Through such obstructions the 
foramen of Majendie may become occluded and dilatation of the third ventricle 
will result ; in the case of occlusion of the foramen of Monro, a distention 
will take place in the lateral ventricle, but increased secretion of serous 
fluid may be sufficient to cause distention of all the ventricles without any 



520 THE NERVOUS DISEASES 0E CHILDREN. 

obstruction whatever. On the other hand, a meningitis may be sufficient to 
occlude these small openings connecting the ventricles with one another, 
and may thus become the actual cause of an internal hydrocephalus. This 
special cause of internal hydrocephalus is of interest only in those compara- 
tively few cases which survive a severe attack of meningitis. A slight in- 
crease of intracranial fluid is present in almost even* case of intracranial 
growth, and is responsible for many of the general cerebral symptoms ac- 
companying such diseases ; but as we said above, the recognition of tumor 
is so much more important than the diagnosis of an accompanying hydro- 
cephalus that the latter loses very much in importance. 

The symptomatology of the acquired form of internal hydrocephalus dif- 
fers in no respect from that of other chronic forms except that the symptoms 
develop with some suddenness ; vertigo, dizziness, stupor, coma, convulsions, 
contractures, and amblyopia, preceded possibly by a condition of hemianopsia, 
are the symptoms which should be looked for in these patients. It stands to 
reason that if the internal hydrocephalus originates at the time when the bones 
are still yielding, the injury to the brain and the symptoms resulting therefrom 
will be far less marked than they will be if it occurs after complete ossification 
of all the sutures and bones. After the sutures have been closed the increas- 
ing hydrocephalus may force them open again. I have not observed this in 
any of the primary forms of hydrocephalus, but have seen it occur in a child 
of four years, who died of cerebellar tumor with enormous internal and ex- 
ternal hydrocephalus. The acquired internal hydrocephalus runs very much 
the same course as the chronic form, and is very apt to terminate fatally in a 
few weeks or months. Moderate cases may survive for a longer period of time. 

The diagnosis of all forms of internal hydrocephalus de- 
pends entirely upon the association of an enlargement of the 
cranial cavity with symptoms which point to an increase of 
intracranial pressure. The exact form of hydrocephalus, 
whether it be congenital or acquired, can be diagnosticated 
only with reference to the history of the individual. In en- 
deavoring to determine whether the hydrocephalus is in- 
ternal or external, we should remember that the external 
hydrocephalus is more apt to be congenital and to be asso- 
ciated with symptoms of defective mental development : and 
furthermore, that bulging of the frontal or occipital bones 
only accompanies internal hydrocephalus; whereas, a gen- 
eral enlargement of the skull in every diameter, with bulg- 
ing fontanelles and sutures forced apart, indicates the pres- 
ence of external hydrocephalus, which is in most cases as- 
sociated with distention of the ventricles as well. But in 
many children the distinction between external and internal 
hydrocephalus is difficult to make, for the internal hydro- 



H YDR O CEP HA L US. 5 2 I 

cephalus may have so thinned out the cerebral substance as 
to bring the ventricular fluid very near the surface. 

Further difficulties of differential diagnosis which arise 
occasionally are to distinguish from hydrocephalus the en- 
largement of the skull due to rickets, to syphilis, or to thin- 
ning of the skull resulting from other causes. In the case of 
rickets, the entire history of the disease will give a clew, and 
other symptoms of rickets will be present and will indicate 
the true nature of the enlargement of the head. But both 
rickets and hydrocephalus are not infrequently associated 
with each other. An increase in the size of the skull may 
simulate the condition of hydrocephalus. Such thickening 
is extremely rare, but is met with in the subjects of hered- 
itary syphilis. I have seen this but once, in a boy, aged six 
years, in whom there were other signs of hereditary syph- 
ilis ; yet the skull is never so much enlarged as in the ex- 
treme cases of hydrocephalus, and generally impresses one 
as being unusually hard. 

Prognosis. — The prognosis of hydrocephalus is always 
serious, but depends greatly upon the original cause. Fort- 
unately a goodly number of children with varying degrees 
of hydrocephalus not only live a number of years, but get 
well. The congenital cases are more rapidly fatal than the 
acquired form, and the prospects of a normal mental de- 
velopment are always slight, though remarkable exceptions 
may occur. Measurements of the skull taken at regular 
intervals will enable the physician to gauge the rate of in- 
crease in the accumulation of fluid, and will help him to 
prognosticate the future course of the disease. 

Treatment. — In the milder forms of hydrocephalus, the 
iodides and the preparations of iron will do good service. 
In the severer forms of hydrocephalus the attempt should be 
made to absorb some of the fluid which is present in excess 
by the use of the mercurials and diuretic measures. I can- 
not state that I have seen any favorable results following 
upon this course of treatment, though some temporary re- 
lief has been afforded in a few cases. The treatment is. 
however, a thoroughly rational one. In almost every form 
of acute and increasing hydrocephalus, however little faith 
he may have in the efficacy of the method, the physician or 



522 THE NERVOUS DISEASES OF CHILDREN. 

surgeon will be tempted to puncture the fontanelles, and 
thus drain away some of the fluid. The fluid is replenished 
so rapidly that very little good can be effected in this way, 
and if it is drained off suddenly the danger of collapse is 
very great. But with the present antiseptic principles of 
surgery there is no good reason why this procedure should 
not be adopted. Instead of draining away a large quantity 
at one time, it seems much more rational to attempt some 
method of gradual drainage. 

In the endeavor to drain away hydrocephalic fluids, several methods have 
been suggested. Keen has given directions for reaching and draining the lat- 
eral ventricles, but his successes have not been such as to warrant a repetition. 
More recently Quincke has suggested puncture of the spinal membranes 
in the lumbar region between the vertebrae. According to Quincke's account 
the operation is attended by little danger and temporary relief is afforded by 
this operation.* Browning has given this method a trial, but his success has 
not been very great. Recent experiences in cranial surgery lead me to think 
that every surgical procedure would be attended by considerable danger. The 
question of slow drainage is a very difficult one. The attempt was made by 
Dr. Gerster to empty a large cyst of the brain by the use of horse-hair, in one 
of my cases, but the patient unfortunately succumbed. While other methods 
could readily be suggested, I believe that the surgeon will have but few suc- 
cesses to record in this special field. Every other treatment is of little avail, 
including the application of elastic bands about the head, and of compression 
of various kinds. I have myself made patient efforts to reduce the skull by ap- 
plying bandages more or less firmly around the head, but the result has in 
every instance been equally unsatisfactory. If the skull is firmly compressed 
the internal pressure is naturally increased, and if the compression is not firm 
enough the bandages will do no good. 



BIBLIOGRAPHY. 

Anton: Med. Jahrbucher, 1888, N. F. III., Heft 4. 

Browning: Journal of Nervous and Mental Disease, 1894, p. 651. 

Keen : Medical News, 1889. 

Quincke : Meningitis Serosa, Volkmann's Sammlung, No. 67, Berl. klin. 

Wochenschr., 1891, Nos. 38 and 39. 
Wyss : Correspondenz Blatt f. Schweyer Aerzte, 1893, No. 8. 

* Quincke's method deserves further trial ; it has a diagnostic value also, for the 
examination of the fluid withdrawn may reveal the true character of the morbid process. 



CHAPTER XXVIII. 

INFANTILE CEREBRAL PALSIES. 
(SPASTIC HEMIPLEGIA, DIPLEGIA, PARAPLEGIA.) 

The researches of recent years have brought to light 
many new and interesting facts regarding the cerebral pal- 
sies of children. For the sake of convenience Ave may 
divide them into three groups according to their onset — 
during the intra-uterine period, during labor, or after birth. 
A clearer understanding of all these disorders will be 
reached by discussing them together, comparing and con- 
trasting the chief features of the one series with those of the 
other groups. 

The subject is not a new one, yet the various forms of cerebral palsies did 
not attract the attention which they deserved until a decade ago. A clear 
account of their clinical symptoms was given by Von Heine in the second 
edition of his book on " Infantile Spinal Palsies " (i860). Heine distin- 
guished between the typical cases of infantile spinal paralysis and those of a 
purely cerebral character. Before his day some of the older French authors 
■ — Cazauvielh, Breshet, Cruveilhier — had studied the atrophic changes in the 
brains of children who had been afflicted with various forms of palsy. These 
writers were interested in the anomalies of brain structure rather than 
in the clinical features presented by such cases. In 1842 Henoch published 
a dissertation entitled " De Atrophia Cerebri," in which he described the 
brain condition associated with infantile cerebral hemiplegia. The chief ad- 
vances were due, however, to Cotard, a pupil of Charcot, who in 1868 pub- 
lished " A Study on the Partial Atrophy of the Brain," in which he analyzed 
the different morbid processess to which such atrophy might be due. Cotard 
was first also to recognize the importance of traumatic encephalitis as a factor 
in the causation of these conditions. A special study of brain defects was 
made by Heschl, who introduced the term " porencephaly," and by Kuiulrat. 
who in 1882 published a monograph on this same subject, in which he dis- 
tinguished between congenital and acquired porencephaly, and attempted to 
explain all these conditions by attributing them to a form of anaemic necrosis. 
In 1883 Audry published a collection of one hundred and three cases of this 



524 THE NERVOUS DISEASES OF CHILDREN. 

peculiar condition. Lobar sclerosis, a condition to which Cotard had called 
attention, was made the special subject of papers written by Bourneville, 
Richardiere, Jendrassik, and Marie. The last author introduced a decided 
advance in the discussion of the subject by connecting this lobar sclerosis 
with changes in the blood-vessels, and still the character of the initial affec- 
tion remained rather doubtful. Quite apart from all these French and 
German authors, Little, in 1853, had studied carefully the spastic palsies and 
rigidities of children, and showed that they were largely due to protracted 
labor and premature delivery. The conditions due to these two causes have 
since been known, particularly among German and French authors, as " Little's 
Disease." 

A fresh impetus was given to the study of all these palsies by the lecture 
of Striimpell, delivered in 1884, who declared that acute infantile spastic 
hemiplegia was due to a primary acute encephalitis affecting the gray matter 
of the cord. He insisted upon an analogy between this acute cerebral and 
the acute spinal palsies, and in order to fortify the resemblance between the 
two sets of disease, proposed the term " polio-encephalitis " as the cerebral 
counterpart of " polio-myelitis." The theory advanced by Striimpell was 
ingenious ; the more the pity, that it has not as yet been satisfactorily estab- 
lished, although so grave and able a writer as Leube, states that " hemiplegia 
in young children should in the first instance be considered to be the result 
of a former acute encephalitis." The truth is, that encephalitis is the rarest 
cause of these palsies, and should not be considered at all, unless every other 
morbid state can be safely excluded. But of this later on. 

Striimpell 's lecture was the starting-point of the innumerable studies 
which have led to the clearer recognition of the true pathology of these 
disorders.* 

The brain palsies of children are far more frequent than 
they are generally supposed to be. In the Hospital for 
Ruptured and Crippled in this city, Dr. Townsend, at my 
suggestion, was kind enough to tabulate the cases of spinal 
and cerebral infantile palsies which presented themselves 
for treatment at that institution for a definite period of time. 
His statistics show that during that period ninety-one 
patients with infantile cerebral palsies, and one hundred and 
forty-two cases of infantile spinal palsies were received for 
treatment. I have, during the last five years, seen over 
three hundred cerebral spastic palsies, but this may be 
due to the fact that many physicians knowing my interest 

"Among the many writings the most prominent are those of Bernhardt, Wallenberg, 
Kast, Moebius, Feer, Freud, and Rie, in Germany; of Marie, Cotard, Audry, Gibbot- 
teau, in France; of Ross, Hadden, Gowers, and Ashby, in England ; in this country 
numerous articles have been contributed to this subject by Weir Mitchell, Sarah 
McNutt, Lovett, Osier, Peterson, Sachs, Starr, and others. 



INFANTILE CEREBRAL PALSIES. 



525 



in the subject were at special pains to refer such cases to 
me. 

Whatever the time or manner of origin may have been, 
all cases of cerebral palsy in children are so similar in many 
respects that we may attempt to study the symptoms com- 
mon to all. These various forms of cerebral palsies are 
observed most frequently in the 
earlier years of life, from the time 
of birth up to the age of ten years, 
and even later, but by far the great- 
er majority of them occur during 
the first three years of life. The 
paralysis is of the distinctly spas- 
tic order and according to the dis- 
tribution of the palsy we may dis- 
tinguish between a hemiplegia and 
diplegia (double hemiplegia) or 
paraplegia. Monoplegias are rela- 
tively rare. The rigidity of the 
muscles, the contractures resulting 
from the same causes, and increase 
of all the deep reflexes are the con- 
stant accompaniment of these pal- 
sies. Coma and convulsions occur 
in the initial stage of the acute 
forms, and the convulsions at least 
are often repeated during the later 
stages of the disease. The cere- 
bral palsies of children are more 
commonly associated with coma 
and convulsions than are those of 
the adult ; the former are generally 
due to cortical processes, the latter 
to intra-cerebral conditions. The frequent repetition of 
convulsions is equivalent to the establishment of an epi- 
lepsy which may continue throughout life, and the same 
changes which have caused the epilepsv may also be re- 
sponsible for the defective mental development, which may 
rano-e from weak-mindedness to marked imbecility and com- 
plete idiocy. Disturbances of motion, associated, ataxic. 




Fig. 138 — Left Hemiplegia. 
Onset in Second Year. Con- 
tractures of arm and fingers. 
(See also Fig. 75.) 



526 THE NERVOUS DISEASES OF CHILDREN 

athetoid, choreiform, and even cataleptic movements, occur 
quite often in connection with these diseases. All of them 
may be grouped under the general heading of post-para- 
lytic (not necessarily post-hemiplegic) disturbances. Apha- 
sia, so common in apoplectic disorders of the adult, is a 
rarer complication in infantile palsies. 

Among the negative symptoms which are of the greatest 
importance in attempting a differential diagnosis between 
the cerebral spastic and the spinal palsies, we may mention 
the entire absence of changes in the electrical reactions and 
the development of only slight atrophy in association with 
the palsy. Disturbances of sensation are rare, but as these 
are absent in the spinal forms as well, they help little in 
attempting a differential diagnosis between these two im- 
portant series of diseases. 

Let us now take up the symptoms seriatim, and in doing 
so we shall be able to develop the many interesting features 
of infantile cerebral palsies. 

Distribution of Paralysis. — The following analysis of 225 cases is 
based upon the collections made by Peterson and myself : 

Males. Females. Total. 

Right hemiplegia 51 30 81 

Left hemiplegia 40 35 75 

Diplegia 21 18 39 

Paraplegia 22 8 30 

Total 134 91 225 

Monoplegias are so rare that they scarcely enter into the consideration of 
the problem. From the above statistics it will be inferred that right and left 
hemiplegias are more frequent that the bilateral forms of palsy, which is 
equivalent to saying that unilateral cerebral processes are more common than 
double cerebral lesions. There is no difference more striking between the 
adult and the infantile palsies than that implied in the relatively large per- 
centage of cases of cerebral diplegia and paraplegia. In the adult a bilateral 
cerebral lesion is a great rarity, but in children diplegias constitute about 
fifteen per cent, of all the cases, and paraplegia about fourteen per cent. 
This difference between the adult and infantile brain is due first of all to the 
fact that both hemispheres in children are exposed to the same external in- 
juries at birth, and even if the disease is acquired later in life the two hemi- 
spheres seem often to be affected simultaneously, and much more frequently 
than in later years. 



INFANTILE CEREBRAL PALSIES. 



527 



In my own statistics boys were affected a little more often than girls, but 
the difference is hardly great enough to be made much of, and Gowers has 
found just the reverse to be true. Osier found that girls and boys were 
affected in about equal proportion. 

The onset of a cerebral palsy will vary naturally according to the three 
groups which we have established. It would seem entirely superfluous to 
make any special remarks regarding the time at which the prenatal palsies 
begin. It might be sufficient to say that they begin during the intra-uterine 
period, and that such children are 
born palsied. It is a matter of fact 
that the symptoms of such intra- 
uterine cerebral defects are not al- 
ways manifest at birth, and indeed a 
number of months may pass before 
it becomes evident to the physician 
that the child's cerebral condition is 
not a normal one. A very fair per- 
centage of cases which appear to 
begin during the first years of life 
could properly enough be classified 
among the prenatal palsies, and if 
in any child the first symptoms of 
a cerebral palsy are noticed sev- 
eral months after birth, and yet the 
period of labor was entirely normal, 
I should be inclined to classify that 
case rather among the prenatal pal- 
sies than among the birth palsies. 

The birth palsies begin naturally 
enough with the period of labor. 
The history in these cases clearly 
shows that the labor was either ex- 
cessively prolonged, or that an in- 
strumental delivery was resorted to, 
in which case the brain has evident- 
ly suffered mechanical injury. Pre- 
mature delivery is responsible for 
many cerebral palsies ; but the symptoms may not be fully developed until 
months after birth. 

The age of onset in the acute or acquired forms varies considerably, and 
yet the chief statistics agree in this that fully two-thirds of all the cases be- 
gin in the first three years of life. Some maintain that by far the largest 
proportion begin during the first year, but I am not inclined to accept this 
view, since such a list would include a large number of cases which are more 
properly congenital, although the symptoms do not become manifest until 
some time after birth. Among a total of one hundred and forty eases. Pe- 
terson and I found that but five began in the eighth year, and four eases 




Fig. 139. — Right Hemiplegia, with Con- 
tractures and Retarded Growth of Arm. 
Onset of disease at eight years of age, 
following typho-malarial fever. 



528 THE NERVOUS DISEASES OF CHILDREN. 

between the ages of fourteen and fifteen years, but these were cases of hemi- 
plegia, and it is of some interest to note that no patients with diplegia or 
paraplegia were observed after the third year, showing that the causes which 
lead to double cerebral lesions, and possibly the condition which permits 
double lesions, cease with the very earliest years of life. The majority of 
diplegias and paraplegias are either congenital or occur during the first year, 
which can be easily explained by the fact that the traumatic injuries during 
labor are largely responsible for these special types. 



Etiology. — The true etiology of the prenatal cerebral 
palsies is often rather mysterious. We have to resort to 
vague statements that they are due to hereditary taints, and 
as a matter of fact such palsies occur frequently enough 
in families with a decided history of neurotic taints of 
one kind or another, with a history of hereditary epilepsy, 
or of hereditary insanity. I have a number of times ob- 
served such children, in whom an hereditary taint was pres- 
ent in the families of both father and mother. The next 
most important factor is unquestionably the occurrence of 
some traumatic injury to the mother during the period of 
pregnancy. This was to be traced distinctly in a number 
of cases of my own. The manner in which such trauma- 
tism may do actual injury to the brain of the child, was 
clearly shown by a case which was cited by Cotard. The 
mother of the child sustained a blow against the abdomen 
during pregnancy. The child, which was still-born three 
months after the injury, exhibited extreme contractures of 
both left upper and lower extremities. On examination of 
the brain an old lesion was discovered in the right hemi- 
sphere in the vicinity of the lateral ventricle. 

Illness of the mother during pregnancy, exhausting 
fevers, particularly such as accompany pneumonia and 
typhoid, ursemic convulsions, and severe fright, are other 
causes which have been made out in cases of prenatal 
palsies.* 

* While revising this chapter my attention was called to a report by Dr. Osier (in 
Teratologia, vol. ii. , Xo. i), of a woman, aged twenty-three years, who died of ty- 
phoid fever. " The uterus contained a foetus, apparently about six months old. . . . 
The brain was very soft ; the right hemisphere was normal, but in the left . . . was 
a cavity with ragged, irregular walls, containing a large, recent clot, which had broken 
through the ganglia into the lateral ventricle of the same side. ... No special 
changes were noted in the arteries." 



INFANTILE CEREBRAL PALSIES. 529 

Hereditary syphilis does not play the important part in 
the etiology of intra-uterine palsies which has been as- 
signed to it by some authors. As a matter of fact I have 
been able to convince myself of the influence of syphilis in 
but a single one of the many cases which I have seen. 
The fact that syphilis of the parents so frequently leads to 
still-births may account in a measure for the small part 
played by this special affection in these diseases. 

The etiology of birth palsies is a very simple one. It is 
surprising to note how much pressure the brain and skull 
will tolerate without injury, but it is natural that harm 
should occasionally be done. Asphyxia at birth has since 
Little's day been considered a most potent factor. My 
own studies in this matter, which were based upon a very 
careful collection of statistics, have proved that tedious 
labor is a more frequent and a more disastrous factor than 
instrumental delivery. Moreover, these birth palsies occur 
most frequently in first-born children. 

The prolonged compression of the skull during the last months of preg- 
nancy would seem to have exercised considerable influence in this direction, 
but, of course, the chief damage done is done during the period of actual 
labor. If physicians were more confident of the safety of the forceps and of 
their own skill to apply the same, protracted labor would not be as powerful 
an etiological factor as it is at the present time. There is, therefore, a distinct 
inference to be drawn from these facts, and a word of warning should be ut- 
tered to the obstetrician that, other things being equal, and, above all, the life 
of the mother not being in danger, it is wise to curtail the period of labor as 
much as possible, and not necessarily to wait until the child's heart action be- 
comes feeble. Many children might have escaped idiocy and epilepsy if the 
period of labor had been properly managed. 

Peterson is of the opinion that some, though a very small percentage (of 
birth palsies) may be due to cord lesions at birth, and not to cerebral lesions. 
He refers to the researches of H. R. Spencer, who among one hundred and 
thirty still-born children, found hemorrhage into the spinal canal and cord 
in thirty. Little suspected that " spinal meningitic and myelitic affections 
may play a considerable part in the phenomena of spastic rigidity ; " he also 
refers to a case of spastic rigidity, reported by Dr. Marion Sims, in which a 
coagulum of blood was found occupying the whole length of the spine. 

Acute cerebral palsies may be due to a variety of causes. 
The acute infectious diseases play a very important role: 
measles, scarlatina, typhoid, small-pox, and even a tonsillitis 
34 



530 THE NERVOUS DISEASES OF CHILDREN. 

have been the precursors of such palsies. The same have 
also occurred after pneumonia and whooping-cough, but in 
the case of the latter it is questionable whether the palsy 
is not due to mechanical injury during a spasm of cough- 
ing rather than to the effect of the toxic agent. Fright — 
which Freud has interpreted to be equivalent to a psychic 
trauma — is an occasional cause of acute cerebral paralysis, 
but actual traumatic injury to the skull is a much more 
powerful factor. These palsies also occur after simple or 
cerebro-spinal meningitis, after an exhausting gastroente- 
ritis, and after other slight fevers ; but, of course, there is 
always the danger of making a post hoc a propter hoc. 
Among ninety-one cases of acquired cerebral palsy the ex- 
act cause could not be ascertained in twenty-seven. 

There has been a tendency, particularly among French 
authors, to claim that all cases of acquired cerebral palsy 
were due to acute infectious diseases. This seems to me 
to be straining the point altogether too much, nor is there 
any evidence that acute cerebral palsy in itself represents an 
acute infectious disease, as Strumpell claimed some years 
ago. As a matter of fact only .relatively few of the cases 
of acquired cerebral palsy begin with fever, coma, and 
convulsions, the very symptoms which Strumpell thought 
most characteristic of polio-encephalitis. There is also no 
evidence that this acute brain trouble affects several mem- 
bers of one family at one and the same time ; nor have cere- 
bral palsies occurred during epidemics of infantile spinal 
palsy. 

The importance of convulsions as an etiological factor in acute cerebral 
palsies has given rise to some discussion. Freud and Rie are of the opinion 
that convulsions denote the onset of the cerebral process, but that they never 
hold a causal relation to the palsy which results from the cerebral lesion. I 
grant that in a very large number of cases this statement of the German 
authors is tenable, but in many others the palsy seems to be a more or less 
immediate result of the convulsive seizure. This view has been urged by 
Osier and myself. Anyone who has observed the marked disturbances of 
circulation at the acme of convulsions can readily conceive how easily a 
blood-vessel could burst during this period as a result of excessive stasis. 
This does occur, moreover, not only in children, but even in adults. I have 
the brain of a girl aged seventeen, who had had chronic epilepsy for years, but 
whose general health was not affected by the convulsive seizures ; after the 



INFANTILE CEREBRAL PALSIES. 



531 



last attack which she had she became somnolent, comatose, and died after 
three days. Her brain was covered by a large subpial extravasation, which 
almost completely covered the entire left hemisphere and part of the right. 
If such an occurrence can take place in the brain of a girl of seventeen 
there is no sufficient reason why similar accidents of lesser extent should 
not happen frequently in the case of children. Ashby, not long ago de- 
scribed the brain of a child, twelve years of age, in which a number of old 
cysts were found which were probably the result of hemorrhages occurring 
during convulsions. There is clinical evi- 
dence also which cannot be neglected 
which tends to show that the convulsions 
constitute a decided danger to the child, 
and mark a turning-point in its entire 
career. I have still under my observation 
a child, now nine years of age, which I 
knew from the time of its birth, and which 
was entirely normal until the age of fifteen 
months. It had begun to stand, to walk 
a little, and had acquired some speech. 
It was in every way a healthy and men- 
tally vigorous child. At that age it was 
stricken down with chicken-pox, and had 
a single convulsive seizure, with a marked 
rise of temperature at the onset of this 
acute infectious trouble. From that time 
on the child's mental condition changed ; 
it lost its speech, and to this day, although 
it has grown physically, its mental condi- 
tion is that of complete idiocy. It would 
be a very remarkable instance indeed if 
an acute cerebral process had come on 
at exactly the same time as the chicken- 
pox. 

The Form of Palsy. — Hemiplegia, 
diplegia, and paraplegia are the usual 
forms of cerebral paralysis in children. 
Monoplegia, which we might expect on 
theoretical grounds, is, as a matter of fact, 
extremely rare. (Figs. 1 38-141.) 

The leg evidently recovers very much more quickly than the arm, as in 
adult hemiplegia, and for this reason it is a very great rarity to rind a mono- 
plegia of the leg w T ith few symptoms in the arm of the same side. Under 
the heading diplegia we may classify all those cases in which both halves of 
the body have been involved, and it is better to attach the greatest impor- 
tance to this bilateral character of the palsy, even though the palsy be incom- 
plete, for the bilateral affection points to a double brain lesion, and that is 
the salient point in every such patient. In diplegia the legs may often be 




Fig. 140. — Congenital Diplegia — 
" Frog Girl." Double talipes 
equino varus ; athetosis of left 
hand ; right arm also weak ; in- 
telligence good. 



532 THE NERVOUS DISEASES OE CHILDREN. 

much more affected than the arms, and some authors might be inclined to 
classify such palsies under the category of paraplegia. In a number of in- 
stances there has been a very complete spastic paraplegia associated with 
athetoid or other disturbances of motion in the upper extremities. This 
proves that the upper extremities were at one time involved, and for this rea- 
son I prefer to denote such conditions as an incomplete diplegia, or diplegia 
with partial recovery. Diplegias or paraplegias, in short double cerebral 
palsies, are, in the large majority of instances, due to a prenatal lesion, or to 
traumatism during labor, but I have been able to satisfy myself over and 
over again that both diplegias and paraplegias occur in the acute cerebral 
cases, and for this reason, in addition to others, I must protest against Striim- 
pell's views of making infantile hemiplegia synonymous with the acute cere- 
bral palsy of children. 

The involvement of the face is a matter of some interest. In children that 
are observed long after the onset of the disease the face appears to be entirely 
normal, but in fully twenty per cent, of the acute forms which I have had an 
opportunity of examining, facial palsy was present in the earlier stages of the 
disease. I have never observed a double facial palsy in cases of diplegia. 
The reason of this is not far to seek, for the peculiar position of the facial 
centre is such that freshly exuded blood would not be apt to adhere to this 
part of the brain. Since the publication of my own articles on this subject, 
and those of Freud and Rie, Koenig has called attention to the occurrence 
of mimetic facial palsies in children. The cases which he cites are very con- 
vincing, but this special form of facial palsy is surely very rare, or else it 
would scarcely have escaped the observation of so many authors. 

Aphasia is often associated with acute cerebral palsy. It is invariably mo- 
tor, not sensory in character. Of course aphasia will be developed only in 
those children who have acquired articulate speech before the onset of the 
cerebral palsy. This excludes from the list all diplegias and paraplegias 
which have come on before or during birth. There may be defective de- 
velopment of speech, but no aphasia in any true sense of the word. I have 
the records of at least seventeen children with hemiplegia and undoubted 
aphasia. Of these seventeen, ten occurred with right hemiplegia, and seven 
with left hemiplegia. Eight of these seventeen were observed by me in pri- 
vate practice, and of these eight, five had been distinctly aphasic, and three 
of the five were cases of left hemiplegia. It is of interest to note the relatively 
large proportion of aphasia in children with left hemiplegia. As we grow 
older the left hemisphere evidently obtains the upper hand, and after the 
earlier years of life we become, for all practical purposes, more and more 
left-brained. Bernhardt has come to the same conclusion that aphasia in 
children accompanies right as well as left hemiplegia. Osier has not ob- 
served this equal distribution of aphasia. (For further remarks on aphasia, 
see Chapter XXIV.) 

Inasmuch as the cerebral palsies are due to lesions which involve other 
parts of the brain as well as the motor areas, it is not unreasonable to look 
for additional symptoms in these cases. Thus Freud was the first to calL 



INFANTILE CEREBRAL PALSIES. 



533 



attention to the fact that hemianopsia was at times associated with the brain 
palsies of children. 

Disturbances of sensation are so rare that they can practically be disre- 
garded. 

Next in order of importance to the form of palsy are the 
rigidities and contractures which regularly accompany 
these cases. Some degree 
of contracture is present in 
fully seventy-five per cent. 
of the diplegias and paraple- 
gias. The contractures oc- 
cur early after the onset of 
the palsy, and evidently are 
developed much more easily 
than in the cerebral palsies 
of the adult. It is interest- 
ing in this connection to re- 
fer to the case of Cotard, in 
which the palsy was due to 
an intra-uterine lesion, and 
the child was born with con- 
tractured extremities. The 
contractures may vary great- 
ly in degree and in the num- 
ber of joints affected. The 
flexors and pronators of the 
arm, the flexors of the legs 
and of the feet, are most fre- 
quently affected. In the cases 
of diplegia and paraplegia 
there is, in addition, a con- 
tracture of the adductors of 
the thighs, which is respon- 
sible for the peculiar cross- 
legged position of the legs 
and for the cross-legged progression if the child is at all able 
to walk. All these contractures give the child a characteris- 
tic position and gait by which we can recognize the trouble 
at first sight. Pes equinus or pes equino varus is the most 
common deformity of the foot. In a few cases an equino 




Fig. 141. — Case of Spastic Diplegia. 
Attempt to walk ; cross-legged pro- 
gression ; rigidity and paralysis of legs 
and of right upper extremity ; left up- 
per extremity weak. 



534 THE NERVOUS DISEASES OF CHILDREN. 

valgus is present. If the upper extremity is contractured 
the arm is, as a rule, in close juxtaposition to the trunk, 
flexed at the elbow, and the hand is generally in a position 
of extreme flexion, the fingers often being firmly pressed 
into the palm of the hand. (See Fig. 139.) 

The gait varies much and is dependent both upon the paralysis and the 
degree of contracture. Many cases of hemiplegia in children have exactly 
the same walk as the adult hemiplegic patients have, but the peculiar cross- 
legged progression in cases of diplegia and paraplegia is characteristic of 
these infantile palsies, and has no counterpart in the cerebral palsies of the 
adult. In one case the contractures of the hip and knees were so extreme 
that the girl, who was otherwise well developed and bright, could walk only 
by skipping in the manner of a frog. She was baptized in my clinic as the 
" Frog girl." (Fig. 140.) 

The reflexes are almost invariably exaggerated, as can be expected from 
the fact that the lesion is in the first division of the motor tract. In a few 
instances only have I found the knee-jerk either normal or diminished, for 
reasons which I have been unable to satisfactorily explain, but suspect that 
in such cases there must have been some involvement of the gray matter. 
In one child which I have seen during the past year at my clinic the reflexes 
were increased in the upper extremities, and decreased in the lower, although 
the case was one of right hemiplegia ; on closer examination we discovered 
that two years after the onset of the cerebral palsy the child suffered an at- 
tack of polio-myelitis involving the right leg. This is, by the way, the only 
patient I have seen with a spinal lesion complicating cerebral disease. In 
some cases the reflexes appear to be absent, but this is generally due to a 
very marked contracture of the opposing muscles, which do not permit the 
excursion of the leg. Under such circumstances, however, even though the 
knee-jerk be wanting, the anterior thigh muscle can be seen to contract upon 
the tapping of the tendon. The ankle clonus and the triceps reflex are often 
inhibited for the reasons just stated. In other individuals, the reflexes are 
usually so much exaggerated that a single tap of the tendon is sufficient 
to produce clonic contractions of the muscle. 

The post-paralytic disturbances of motion constitute a very 
characteristic feature of cerebral palsies in children, and 
they follow with much greater regularity upon the infantile 
cerebral lesions than they do upon the brain lesions of the 
adult. That they are by no means uncommon in the brain 
lesions of the adult, I had occasion to observe several 
times, notably in an old woman, with a lesion in the crus, 
whose paralyzed arm performed the most violent ataxic 
movements. In children these post-paralytic disturbances 
of motion occur in fully one-third of all the cases. 



INFANTILE CEREBRAL PALSIES. 535 

From a collection of statistics based upon one hundred and fifty-six cases 
of hemiplegia, and thirty-nine cases of diplegia, it was evident that athetoid, 
choreiform, and associated movements, are the most frequent disturbances 
to be noted. The choreiform and athetoid can generally be differentiated 
from one another, but in some cases there may be a combination of both. 
The athetoid movements are of especial interest, inasmuch as they are ob- 
served almost invariably in connection with the cerebral palsies, and it is 
questionable whether athetosis ever occurs as an independent disease, as was 
claimed by Hammond, who, however, deserves all the credit for the first vivid 
description of these peculiar movements. 

The associated movements are often developed to a startling degree, the 
paralyzed hand imitating all the movements of the normal hand. Thus a 
patient who is asked to button or unbutton his clothes, will imitate all the 
movements of the sound hand, by the paralyzed hand, although the latter 
may be held in mid-air. 

The choreiform movements occurring after cerebral pal- 
sies deserve some special notice, for such conditions are at 
times mistaken for cases of ordinary chorea. I have been 
consulted in not a few instances for what was supposed to be 
incurable St. Vitus's dance, which on examination has proved 
to be a form of infantile cerebral palsy, in which the palsy 
had largely disappeared, but the choreiform movements 
were left as evidences of the former more serious dis- 
turbance. In such children the presence of paralysis or of 
contractures, however slight, and the exaggeration of the 
reflexes in the choreic extremity, will be sufficient to indi- 
cate the nature of the choreic movements. 

Atrophy of the muscles plays an entirely negative part in the vast majority 
of infantile spastic palsies. Quincke, Borgherini, Darkschewitsch, Eisen- 
lohr, and others, have proved beyond a doubt that a cerebral muscular atro- 
phy does at times occur, but it is still questionable whether this is due to 
a lesion of the trophic centres in the brain, or whether the spinal gray mat- 
ter has not in some way been involved. I have observed a considerable atro- 
phy of the muscles in a few cases of diplegia and paraplegia. In one patient 
with paraplegia the atrophy was so extreme that the case would have ap- 
peared to have been one of spinal palsy if the presence of the reflexes and 
the electrical reactions, as far as any could be elicited, together with the en- 
tire history of the disease, had not pointed unmistakably to a cerebral lesion. 
While a true muscular atrophy does not occur, as a rule, the affected limb re- 
mains stunted in growth, and after a few years is considerably shorter than 
its fellow of the opposite side. A few authors have reported hypertrophy 
of the paralyzed extremities, together with athetosis, but it has not been my 
good fortune to see any such case. 



536 



THE NERVOUS DISEASES OF CHILDREN. 



Remak has reported a case of luxation of the shoulder-joint in an infantile 
cerebral hemiplegia, which he attributes to unequal innervation of the mus- 
cles surrounding the shoulder-joint. 

A distinct asymmetry of the body is present in many of the cerebral pal- 
sies, and to this we may add the asymmetric development of the skull, which 
has been shown to be present, by the careful measurements of Peterson and 
Fisher, in the majority of the subjects of infantile spastic hemiplegia ; but the 
skulls of such children are often not only asymmetrical, but also deformed in 
various ways. Some of them are microcephalic, others leptocephalic, doli- 
chocephalic, etc. Microcephalus is frequent enough to justify me in caution- 
ing the surgeon who may be ready to operate upon all such skulls that a 

small skull may harbor a dis- 
eased brain, and that it is 
well to determine this point 
carefully before attempting to 
open up the skull for the pur- 
pose of giving the brain bet- 
ter opportunity for growth. 

The most serious of 
all the symptoms asso- 
ciated with infantile 
cerebral palsy is epi- 

rlepsy. A number of 
"A^gfcjH children have been 

brought to me with the 
diagnosis of epilepsy, 
pure and simple, in 
which this condition 
was the outcome of 
the same cerebral dis- 
turbance which gave rise to the pals)*. The palsy may dis- 
appear, or may be so slight as to give little inconvenience 
to the child, whereas the epilepsy unfortunately remains. 
This association is so common that I am inclined to the view 
that a fair percentage of the cases of supposed genuine 
epilepsies may be attributed to cerebral lesions which oc- 
curred in early childhood, and have given rise to cerebral 
palsies. I am glad to see that this view has been quoted 
approvingly by a number of authors, among others by 
Freud, who has unquestionably given this entire subject 
the most careful study. A single case will bring out the 
truth of this very clearly. 




Fig. 142. — A Child with Congenital Diplegia and 
a Microcephalic Skull ; Strabismus and Idiocy. 



INFANTILE CEREBRAL PALSIES. 537 

Some years ago I was asked to see a girl, seventeen years of age. A 
number of able physicians had treated her for genuine epilepsy. Upon ex- 
amination of the girl I was astonished to find that the left extremities were 
weaker than the right, and that the deep reflexes of the left upper and lower 
extremities were considerably exaggerated. Upon questioning the mother 
closely I discovered that four years previously the girl had had a slight apo- 
plectic seizure, after which the convulsions first appeared. The palsy had 
diminished to such an extent that everyone would have considered the gait 
of the girl entirely normal. Her mental condition was impaired by the 
enormous doses of bromide which had been administered in the hope of 
curing the epilepsy. Recognizing its organic origin, I decided to discon- 
tinue the bromides, and, as a result, the epilepsy is no worse and no better ; 
but the girl's mind has considerably improved, and the anaemia, which was 
aggravated by the administration of the bromides, has been considerably 
diminished. 

The first epileptic attacks associated with a cerebral palsy may occur very 
soon after the onset of the disease, and may be repeated a number of times 
during the first week or two. After this a lull sets in, but unfortunately the 
attacks often recur after several months, sometimes after a year or more, 
when it is positive that a chronic epilepsy has been developed. According to 
my own statistics, fully forty-five per cent, of all the infantile cerebral palsies 
develop epilepsy. This occurs, furthermore, in about fifty per cent, of the 
cases of hemiplegia, in about thirty per cent, of all forms of diplegia, and in 
about thirty-six per cent, of patients with paraplegia. I have furthermore 
observed that the epilepsy does not necessarily develop according to the 
severity of the palsy, for some of the worst cases of epilepsy I have seen have 
been observed in cases in which the palsy was extremely slight. The corti- 
cal origin of infantile cerebral palsies is sufficient reason for the more fre- 
quent development of epilepsy after infantile than after adult cerebral palsies ; 
and if the character of the palsy is such that the lesion in the child can be 
proved to be capsular rather than cortical, the prognosis as regards epilepsy 
may be considered favorable. Jacksonian epilepsy is present in some of the 
palsies, and, if present, can easily be explained on physiological grounds, but, 
as a matter of fact, it is not observed as commonly as one would suppose, 
for the simple reason that the majority of patients have been examined after 
several years have elapsed, and the unilateral order of convulsions has been 
superseded by a general convulsion. 



Idiocy is developed very often with epilepsy and with 
some forms of cerebral palsy. Every possible variation of 
mental deficiency may be present, from slight mental en- 
feeblement to a complete idiocy. The severer forms are 
oftener present in the cases of diplegia and paraplegia than 
in hemiplegia, and naturally enough, for in the former there 
is a double lesion or disease of both halves of the brain 



538 THE NERVOUS DISEASES OF CHILDREN. 

which will disturb the mental development far more than a 
unilateral lesion would. 

I have found idiocy present in thirty-five per cent, of all diplegias, and in 
sixty per cent, of all paraplegias, while it occurred in but thirteen per cent, 
of the subjects of hemiplegia. It was this very high percentage of idiocy in 
cases of diplegia and paraplegia that forced the conclusion upon me, some 
years ago, that these congenital paraplegias must be of cerebral rather than of 
spinal origin. There is no telling in advance whether, in a given case, idiocy 
will develop or not, for, unfortunately, relatively slight paralysis is sometimes 
complicated by severe idiocy. The lesion may involve the motor area to a 
slight degree only, and may have spent its force upon the frontal lobes of 
the brain. The frontal lobes are supposed to be silent parts of the brain, 
but the defect in intelligence tells a sadder tale of its important functions 
than the palsy does of the functions of the motor centres. 

Various attempts have been made to classify the cere- 
bral palsies of children, and to separate them into distinct 
clinical groups. The classification according to the period 
of development, which we have adopted, is, on the whole, 
the most practical one. The palsies due to intra-uterine 
disease or to arrest of development, and those due to 
traumatism during labor, give a distinct history of early 
onset of all the symptoms. In these children bilateral pal- 
sies (diplegia and paraplegia) are more common than in the 
acute cases, and there is often a very decided defect in 
mental development. The paralysis is not necessarily com- 
plete ; at times it is a simple paresis, at other times rigidi- 
ties and contractures are more prominent than the palsies 
are. English and American authors have not drawn any 
sharp clinical lines, as all these forms, although they may 
vary a little, seem to merge into one another, and are evi- 
dently due to similar morbid processes. Little distinguished 
between rigidities and palsies, and several German authors 
have adhered to this distinction ; but one of the ablest of 
them, Freud, has practically adopted the American point 
of view. Rigidity, contracture, choreic or athetoid * move- 
ments, paresis, paralysis (unilateral or bilateral), constitute 
a progressive series of disturbances due to cerebral lesions 
in children. Each one, or a combination of all of them, may 

* The subject of double athetosis has been studied most exhaustively by Audry, who 
embodied his researches in a monograph, Paris, 1892. 



INFANTILE CEREBRAL PALSIES. 539 

be present in a given case, and may vary according to the 
site and intensity of the lesion. The best proof that all 
these disturbances of motility are closely related is, that in 
certain cases of diplegia there is paralysis in the lower ex- 
tremities and athetosis in one or both upper extremities. 
Double athetosis is evidently a condition closely analogous 
to the double hemiplegia, with less paralysis than in other 
cases. Freud showed that there was a form of acute cere- 
bral palsy with little palsy but distinct chorea ; he described 
this condition as one of choreiform paresis, and recognized 
an early and a late form. Unilateral and bilateral* chorea 
and athetosis may be developed in the place of a palsy, or 
the palsy may come first and the chorea and athetosis may 
represent post hemiplegic disturbances of motion. 

The diplegias and paraplegias are generally due to ar- 
rest of development or to abnormal birth conditions. (A 
family form of diplegia was discussed under the heading of 
" The Cerebral Type of Hereditary Spastic Paralysis.") 
French authors (Charcot, Marie, and others) insist that the 
congenital spastic paraplegia is a spinal affection and due 
to defective development of the pyramidal tract. For a 
time Ross adhered to a similar view ; but the defect surely 
involves the brain in a large majority of instances, or else we 
could not account for the high percentage of idiocy among 
children with congenital paraplegia. It is in this group of 
diseases that thoroughly satisfactory post-mortem examina- 
tions are needed. One case of Foerster and one of my own 
have been relied upon, but to my own case I now give a 
different interpretation (see page 399). 

Morbid Anatomy.— The study of the morbid lesions 
underlying these cerebral palsies in children will be facili- 
tated very much by considering separately the three chief 
groups of cases. A reference to the following table will 
show that in the prenatal cases large cerebral defects are 
often found. Porencephaly is present in its widest sense. 
Half or even more of an entire hemisphere, or of both 
hemispheres, may be wanting, or a considerable portion of 
one hemisphere may be poorly developed ; in such cases, too, 
as a rule, the palsy which exists is but one of a long series oi 
symptoms, among which idiocy is by far the most promi- 



540 



THE NERVOUS DISEASES OF CHILDREN. 



nent. If it were not for the fact that porencephalic defects 
occur more frequently in the motor areas of the brain than 
in any other part, we would scarcely be entitled to associate 
porencephaly more especially with these cerebral palsies. 

Classification of Infantile Cerebral Palsies. 






Groups. 



I. Paralyses of intra-uterine 
onset 



II. Birth Palsies 



Morbid Lesion. 



f Large cerebral defects. (Porencephaly). 
J Defective development of pyramidal tracts (p. 539). 
] I Agenesis corticalis. (Highest nerve elements ra- 
il volved.) 



( ' Meningeal hemorrhage, rarely intra-cerebral 
I hemorrhage. Later conditions : Meningo-en- 
j cephalitis chronica, sclerosis, and cysts ; partial 
(, atrophies. 



III. Acute (acquired) Palsies. 



Hemorrhage (meningeal, and rarely intra-cere- 
bral) ; thrombosis (from syphilitic endarteritis 
and in marantic conditions ; embolism. Later 
conditions : Atrophy, cysts, and sclerosis (dif- 
fuse and lobar). 

Meningitis chronica. 

Hydrocephalus (seldom the sole cause). 

Primary encephalitis ; polio - encephalitis acuta 
(Strumpell). 



In addition to these large defects in cerebral develop- 
ment, other changes occur in the brain which are not quite 
as conspicuous, but are fully as effective in the way of pro- 
ducing serious symptoms and serious conditions during life. 
This condition has been properly designated " agenesis cor- 
ticalis." It implies a defective development of the cellular 
elements of the cortical, and particularly of the pyramidal, 
cells, and is not restricted to any one part of the brain, but 
involves all parts of the hemispheres about equally. This 
condition is met with in the family form of idiocy, com- 
bined with amaurosis (see page 397). 

The morbid lesions in birth palsies have been satisfacto- 
rily cleared up within the past few years. We owe much 
of this knowledge to the successful demonstration by Dr. 
Sarah McNutt of a case in which there was wide-spread men- 
ingeal hemorrhage in consequence of traumatism during 
labor. (Fig. 143.) Dr. McNutt furnished positive proof 
of a condition which Little suspected years ago, in attribut- 



INFANTILE CEREBRAL PALSIES. 



541 



ing these palsies to the difficulties during birth. There 
can be no doubt but that in a vast majority of cerebral birth 
palsies meningeal hemorrhage, more or less diffuse over 
one or both hemispheres, is the direct cause of the disease. 

The failure to recognize this simple condition has been due to the fact 
that the cases examined for the purpose of determining the morbid states 
underlying these palsies have lived for a number of months, or even a year, 
and at the end of that time the character of the initial lesion may not be 
recognized. A very instructive case of this description is the one referred to 




Fig. 143. — Meningeal Hemorrhage at Birth. 

(McNutt.) 



Death on the twenty-second day. 



in a previous chapter (page 400). The child was born asphyxiated after 
forty-eight hours continuous labor pains, and an absolutely dry labor at that. 
From the very first day of its life until the age of six months, when I first 
saw the child, it had innumerable epileptic spells. The child died at the age 
of one year. The autopsy of the brain revealed a wide-spread chronic 
meningo-encephalitis (Fig. 98). This condition was in all probability due 
to a meningeal hemorrhage, though the traces of the hemorrhage had so thor- 
oughly disappeared that it was difficult to prove this with absolute certainty. 

In some instances an original meningeal hemorrhage, particularly if it be 
more or less circumscribed, may lead to the formation of a limited atrophy. 
in which case a condition resembling porencephalus may be the result. A 
few years ago Kundrat described a condition of minute hemorrhages from the 
meningeal veins, which he considered to be caused by a compression of the 
longitudinal sinus, in consequence of the displacement of the parietal bones. 



542 THE NERVOUS DISEASES OF CHILDREN. 

Such hemorrhages Kundrat thought were found rather frequently in the brains 
of healthy children. It is doubtful whether we can connect this form of 
venous hemorrhage with the subject which we are specially considering. 

The amount of damage done during labor can be gauged 
to a certain extent by the symptoms during the first few 
days of life. If there is wide-spread meningeal hemorrhage 
convulsions set in at a very early period ; the child is apt to 
be paralyzed at once, and is also in danger of lapsing into 
coma. Fortunately a number of the severer cases die 
promptly within a few days, but others again survive, and 
not a few of these become hopelessly crippled for life. Ac- 
cording to the amount of injury done, and according to the 
special parts of the brain covered by the hemorrhage, the 
form of the palsy, the degree of mental enfeeblement, and 
the severity of the epilepsy will vary not a little. 

The morbid anatomy of the acute cerebral palsies has 
given rise to the largest amount of discussion. Hemor- 
rhage, embolism, and thrombosis, the conditions which give 
rise, in the vast majority of cases, to the apoplexy of the 
adult, are important factors also in the causation of the acute 
cerebral palsies of children. This conclusion was forced 
upon us and others by a careful review of the autopsies 
made by different authors. 

Peterson and myself analyzed the records of seventy- 
eight autopsies in infantile hemiplegia, as follows : 

Lesions. Number of Cases. 

Atrophy, sclerosis, and cysts (terminal conditions). .... 40 

Porencephalus 2 

Hemorrhage 23 

Embolism 7 

Thrombosis 5 

Tubercle 1 

Total 78 

In 35 cases of hemiplegia hemorrhage occurred in 23, 
embolism in 7, and thrombosis in 5 cases. Professor Osier, 
who examined 90 brains, found a vascular lesion in 16 only, 
and among these 16, 7 were due to hemorrhage, and 9 
to embolism ; but we must not forget that a large number 
of the cystic conditions may be due to hemorrhage or em- 



INFANTILE CEREBRAL PALSIES. 



543 



bolism, and that many a case which is quoted as typical 
atrophy or porencephalus may also belong to the same 
category. There has been some little difference of opinion 
among recent writers on this subject with regard to the 
relative frequency of these various conditions. Some are in 
favor of the greater frequency of embolism ; others, like my- 
self, believe that hemorrhages are most commonly the cause 
of these conditions ; while according to Gowers thrombo- 
sis of the superficial veins is a very noteworthy initial lesion. 
The probability is that all these accidents occur, and that 




Fig. 144. — Cyst formed by Softening of Brain Substance, secondary to Obstruction of* 
the Middle Cerebral Artery beyond the First Branch (to Inferior Frontal Convolu- 
tion). The cyst wall has fallen in from escape of its contents. Child nineteen 
months old. Death seven months after onset of paralysis. (After Ashby and 
Wright.) 

there is no satisfactory reason for claiming more for the 
one than for the other finding ; but with regard to the occur- 
rence of hemorrhages it is fair to inquire what morbid con- 
dition of the blood-vessel leads to the frequent occurrence 
of hemorrhage in children. 



In my endeavors to elucidate this part of the inquiry I have been compelled 
to fall back upon the fatty degeneration of the blood-vessels which Reck- 
linghausen describes in his masterly Monograph, and which he claims is a 
not uncommon finding in the brains of children. For the present we can do 
no better than to adhere to this view of Recklinghausen, and to accept it as a 
partial explanation, at least, of the frequent occurrence of hemorrhage. Some 
cases are of course explained by the more delicate structure of the blood-ves- 



544 



THE NERVOUS DISEASES OF CHILDREN. 



sels in children as compared with the adult. But there is still another striking 
difference between these infantile and adult apoplexies. In the adult the 
majority of hemorrhages occur in the vicinity of the internal capsule. In the 
child they occur in or near the cortex. Mendel has shown that the greatest 
pressure is exerted in the branches of the middle cerebral artery, and that 
any increase of pressure would naturally cause a rupture in one of these. 
We would have to seek some other explanation for the frequent occurrence 
of cortical and meningeal hemorrhages, and perhaps they will be sufficiently 
accounted for by the more delicate structure of these vessels in children. Oc- 
casionally intra-cerebral hemorrhages, embolism, and thrombosis occur in 

















mwm 



^-<»t 










Fig. 145. — Section through Portion of Motor Cortex, removed by Dr. Gerster during 
an Operation for Localized Epilepsy, associated with Old Left Hemiplegia in a Boy- 
Twelve Years of Age. Specimen was stained according to Van Gieson's method. 
The pia, P, which is greatly thickened and altered, dips down between two folds of 
the cortex, C. B, increased number of thickened small arteries ; just above, to the 
right, a large artery with thickened walls. H points to a recent clot. The line, a b, 
denotes the part of cortex examined under higher power and represented in Fig. 146. 



children in exactly the same manner in which they occur in the adult, and 
they give rise to clinical symptoms in nowise different from those of ordinary 
apoplexy. An interesting case of this sort I had occasion to observe in a boy, 
two and a half years of age, who after a simple attack of tonsillitis developed 
right hemiplegia with motor aphasia, without coma and without convulsions. 
The aphasia disappeared after a few days, and the hemiplegia also improved ; 



INFANTILE CEREBRAL PALSIES. 



54! 



within a period of a few weeks the young boy was entirely well, and scarcely 
retained a trace of the apoplectic condition. There was no history of syph- 
ilis in the case, and the entire development and retrogression of the symp- 
toms reminded one of an adult apoplexy. The possibility of thrombosis 
could not be excluded, but whether hemorrhage or thrombosis, there can be 
no doubt about this, that the lesion was intra-cerebral and not cortical, as in 
the majority of cases in children. Since my own case was published, De- 
jerine, in 1891, reported three cases in 
which there were distinct hemorrhagic 
lesions in the vicinity of the larger gan- 
glia. 



Heart disease, rheumatism, 
scarlet fever, and pneumonia, 
are the conditions which pre- 
dispose to embolism. Throm- 
bosis may be suspected in cases 
of children dying of marasmus, 
but under such conditions the 
existence of a palsy, is, as a 
rule, overlooked, and of little 
practical importance. 



Thrombosis may also be the result of 
arterial changes due to hereditary syph- 
ilis, but I wish distinctly to impress upon 
the reader that in these cases of cerebral 
palsies syphilis does surely not play as 
important a role as is imputed to it by a 
few authors, who have generalized from 
the examination of one or two cases. 
Gowers has advanced important reasons 
for the supposition that thrombosis and 
occlusion of the middle cerebral veins is 
a frequent occurrence in children, and 
that some of the cases of infantile hemi- 
plegia would be more likely to be due 
to this condition than to embolism re- 
sulting from endocarditis. He states, 
furthermore, that the thrombosis cannot 
be distinctly demonstrated post-mortem, 
because the thrombosis is, as a rule, 
continued into the sinuses, and a sinus thrombosis would be much more 
striking, and would be apt to conceal the venous thrombosis which led to it. 
Gowers's views seem to me to deserve considerable attention. 
35 




Fig. 146. — Variously Degenerated 
Cells of the Cortex in the vicinity 
of the line a b of the preceding fig- 
ure. Near a, small blood-vessels, 
walls thickened. Large pyramidal 
cells are misshapen, and exhibit 
granular disintegration. Cells di- 
minished in number. (Drawn from 
specimen. ) 



546 



THE NERVOUS DISEASES OF CHILDREN. 



Whatever the initial lesion of an acute cerebral palsy 
may be, if the patient survive a number of years, secondary 
changes may be set up in the brain which will successfully 
conceal the initial lesion. Cysts, large areas of softening, 
atrophy, sclerosis (diffuse and lobar), are a few of the 
changes frequently observed. 

There is no telling in advance of the post-mortem examination, with any 
degree of certainty, which secondary lesion will be found in the brain of a 




Fig. 147. — An Old Hemorrhagic Cyst. The cyst walls have been cut to expose tu- 
mor underneath (compare with Fig. 150). Right hemiplegia at age of six and a half 
years ; death two years later. 

child that has been afflicted with an acute cerebral palsy for a number of 
years. Cysts are so frequent that I have in a number of instances been able 
to predict the presence of this condition, particularly in those cases in which 
there was little idiocy associated with the palsy, and in which everything 
seemed to point to an initial lesion of considerable intensity, but limited in 
extent. And in several other cases that came under my own notice for opera- 
tion the surgeon found a cyst in the motor area, which I had predicted to 
be the probable condition. 

Various forms of sclerosis are among the most frequent 
sequelae of the initial lesions of infantile cerebral palsies. 



INFANTILE CEREBRAL PALSIES. S47 

This sclerosis evidently starts from a focal region and rap- 
idly speads throughout the brain. If we bear in mind that 
the hemispheres are traversed by innumerable fibres which 
are intimately connected with one another, we can under- 
stand why a sclerosis should follow upon a relatively small 
focus of disease, just as secondary degeneration follows 
upon a lesion in the motor area, and affects all the fibres 
transmitting centrifugal impulses. This sclerosis, which 
develops after an initial lesion, is largely responsible for the 
symptoms which are so frequently associated with these 
palsies. I refer to idiocy and epilepsy. 

In the table given at the beginning of this discussion I have, for the sake 
of completeness, inserted chronic meningitis as an occasional cause of an ac- 
quired cerebral palsy. It is not entirely accurate to attribute a cerebral 
palsy to a chronic meningitis, but it is better to say that both the chronic 
meningitis and the cerebral palsy are the result of a cerebro-spinal or of a 
convexity meningitis which occurred early in life, and which the child sur- 
vived. These cases are sometimes to be differentiated from others by the 
persistent paralysis of various cranial nerves in addition to the paralysis of 
the extremities. 

The reader may be surprised to find polio-encephalitis acuta given as the 
very last morbid condition underlying acute cerebral palsy. Strumpell's idea 
was a fascinating one indeed, and it is to be regretted that later post-mortem 
findings did not support his theory. The analogy which this author drew 
between the infantile spinal paralysis and the infantile cerebral palsies was 
based upon clinical resemblance, but we well know that symptoms which are 
scarcely to be differentiated from one another may be due to a variety of 
morbid processes. Striimpell maintained that there was a close clinical and 
pathological relation between polio-myelitis and polio-encephalitis acuta. 
More recently he has modified his views, and now claims that acute encepha- 
litis of the gray, as well as of the white matter, may constitute the basis for 
these acute cerebral palsies in children. The actual proof which Striimpell 
offered for his views was based upon the occurrence of two cases in one 
family, reported by Moebius, in which two children were affected at one and 
the same time ; the one with a typical polio-myelitis, and the other with a 
spastic cerebral palsy without atrophy. This may have been a mere coinci- 
dence, and further evidence must be forthcoming before we can accept this 
as proof of the theory. Striimpell himself refers to two cases in the adult in 
which the diagnosis of hemorrhage was made during life, and in which, at 
the post-mortem examination, acute encephalitis was found. From this we 
may infer the possibility of a similar occurrence in children, but until such a 
condition can be satisfactorily established there is no good reason to accept 
encephalitis as more than the rarest of all causes that give rise to infantile 
cerebral palsies. Before leaving this part of the subject it may be well to 



54-8 THE NERVOUS DISEASES OF CHILDREN. 

suggest to the student that the same lesions, which when affecting the motor 
areas give rise to a palsy, may occur in silent parts of the brain, and that the 
result of such lesions may be a general cerebral disturbance, defective intelli- 
gence, or chronic epilepsy. The fact has been well emphasized by Freud 
who speaks of cases of " cerebral palsy without palsy." 

Differential Diagnosis. — Infantile cerebral palsies 
are frequently confounded with the acute spinal affections. 
The cerebral cases are characterized by the hemiplegic, 
diplegic, or paraplegic form of paralysis, by spastic rigid- 
ities and contractures, by increase of the reflexes, and by 
the entire absence of any considerable degree of atrophy, 
and by entirely normal electrical reactions. Anyone who 
is able to recognize the difference between lesions in the 
first and second divisions of the motor tract will be able 
to distinguish between the cerebral and spinal forms of in- 
fantile palsies. Difficulties may, however, arise in the cases 
of mild types of cerebral palsy in which the spasticity and 
contractures may be but slightly developed, and the re- 
flexes may not be very much increased ; but even under 
such circumstances the hemiplegic distribution of the palsy 
and the entire absence of all changes in the electrical 
reactions will help to demonstrate the cerebral character of 
the disease. In other patients, the presence of athetoid or 
choreic movements in one half of the body, with slight ex- 
aggeration of the reflexes, will indicate a former cerebral 
palsy in which the paralysis has pretty well disappeared. 
Spinal and cerebral palsies may occur at different periods 
of a child's life, but I have seen only one instance of this 
among the large number of cases that have come under my 
notice. The spastic paraplegia due to traumatism during 
labor is to be differentiated from hereditary spastic paraly- 
sis (see Chapter XXII.) and from syphilis of the spinal cord 
(see Chapter XVIII.). 

Although the diagnosis is simple enough, the knowl- 
edge of these infantile cerebral palsies is still so scantily 
spread among physicians that cases coming under this cat- 
egory are frequently overlooked. I would particularly 
urge the examination of patients with peculiar forms of 
athetosis or chorea in order to determine whether these 
are not associated with some traces of a preceding cerebral 



INFANTILE CEREBRAL PALSIES. 549 

palsy. If such children exhibit hemiparesis with slight 
defects in mental development, or with occasional epileptic 
attacks, the true nature of the disease can readily be dis- 
cerned. 

Prognosis. — In the congenital forms of cerebral palsy 
the prospects as regards life and the normal development 
of the child cannot be formulated until after a period of 
some weeks or months. It is a fortunate circumstance on 
the whole that children who come into the world with a 
severe cerebral lesion succumb to it at a very early period, 
but of those who survive a fair proportion develop idiocy 
as well as epilepsy, and are frequently permanently crip- 
pled. If convulsions occur during the earlier weeks of 
life the severity of the lesion may be inferred from this 
fact. If such a child, moreover, show no mental awakening, 
the probability of more or less complete idiocy is very 
great indeed. If, after a few weeks or months, the convul- 
sions are noticeably diminished, if the child shows any ten- 
dency to a tolerably normal use of its legs, and if it begins 
to take notice of its surroundings, a more favorable prog- 
nosis may be given. As long as contractures do not de- 
velop the child may acquire a fair use of its extremities. 
Diplegia and paraplegia are more apt to be associated with 
cerebral deficiency and epilepsy than unilateral palsy is. 

In the acute cases there is much uncertainty for some 
time after the onset of the attack, and no definite statement 
is warranted regarding the permanency of the paralysis, or 
the mental condition of the child until several weeks have 
elapsed, when it will be seen whether there is any tendency 
or not to recovery. If a child with an acute form of infan- 
tile hemiplegia shows some improvement after a few weeks, 
either in the leg or in the arm, and if the speech that was 
lost begins to return, there is every reason to be hopeful 
regarding the ultimate outcome of the disease ; but if weeks 
and months pass without any such favorable change the 
probability of permanent crippling of the mind and body is 
very great. It is, however, a matter of common experience 
that the child may do extremely well for a number of 
months, for a year, or even longer, after an attack, when 
convulsions may reappear and the epileptic habit may be- 



550 THE NERVOUS DISEASES OF CHILDREN. 

come established. I have paid considerable attention tc 
this point, and find that among the cases examined by me 
epilepsy occurred in fully forty-five per cent, of all the 
cerebral palsies, and nearly one-half of the cases of cerebral 
hemiplegia developed epilepsy at a later period. It would 
seem from this that the unilateral brain lesion leads to epi- 
lepsy more frequently than the double lesion does, for ac- 
cording to the same statistics epilepsy is present in about 
twenty-nine per cent, of the diplegias, and in about thirty- 
six per cent, of the cases of paraplegia. My own results 
regarding the development of epilepsy are corroborated by 
the statistics of Gaudard, Osier, and Wallenberg. The 
fact that fewer cases of diplegia and paraplegia survive 
may account for this apparent difference in favor of the 
double lesions. If a child that has passed through an 
apoplectic seizure, and has some form of congenital paraly- 
sis is taken with convulsions, it is more than probable that 
such convulsions are the first of a series that may lead to 
the development of chronic epilepsy, and from this time on 
the prognosis becomes extremely grave. 

Treatment. — The treatment of infantile cerebral pal- 
sies is not altogether devoid of interest. These diseases 
call for deliberate non-interference at the start, and for 
sober judgment in therapeutic matters during the later 
stages. The treatment of the later stages will be prac- 
tically the same whether a case be congenital or acquired. 
If a child but a few days old exhibits a tendency to drow- 
siness or to convulsions its brain has in all probability sus- 
tained a serious injury during labor. It should be kept 
quietly in its crib or bed, and the greatest attention should 
be paid to its nutrition. These children are often unable 
to swallow and unable to suckle at the breast, and for this 
reason must be carefully fed with a spoon. Milk, properly 
prepared, according to the age of the child, is the very best 
nourishment. If there is a tendency to convulsions, or if 
marked convulsive seizures occur, the child may be given 
very minute doses of bromides, or minimum doses of mor- 
phia or chloral. A drop of the solution of Majendie, or a 
grain of chloral per rectum, will be quite sufficient in the 
case of a new-born babe. If convulsions continue in spite 



INFANTILE CEREBRAL PALSIES. 551 

of these measures, inhalations of chloroform may be prac- 
tised, however young the child may be, but of course with 
the greatest possible care. All other measures, such as 
counter-irritation, mustard-baths, and the like, are practi- 
cally useless. 

In the initial stages of an acute cerebral palsy the same 
measures should be employed which the physician would 
resort to in the case of an adult apoplexy, allowing always for 
the difference in the ages of the patients. Absolute rest is 
the first condition of treatment. An ice-bag may be ap- 
plied to the nape of the neck, or to the head, and if it do no 
good, it will at least do little harm. The use of ergot or of 
the nitrite of amyl, as has been advocated in adult apoplexies, 
I cannot favor. The fewer drugs that are administered the 
better it will be, and the physician will do well to limit 
himself entirely to the administration of calomel in doses suf- 
ficient to procure free purging of the bowels, and small doses 
of bromide, which may be given in order to secure rest for 
the disturbed brain. At a later period the bromides may 
be combined to advantage with the iodides, but in every 
case in which the digestion or the general nutrition suffers 
from the administration of these drugs, their use should be 
prohibited for the time being ; for the good that they can 
effect will not counterbalance the evils that follow upon a 
gastritis and its attendant malnutrition. 

As soon as the symptoms of the initial period have 
passed, the physician is compelled to prescribe some form 
of treatment for the paralysis and the other symptoms of 
the disease. The paralysis is the natural result of the lesion, 
and cannot of course be removed by any therapeutic meas- 
ures. It is well to explain this distinctly to the parents 
and relatives, and to state at once the chronic nature of 
these troubles, and the length of time that will probablv 
elapse before any great change can be expected to take 
place. As soon as the active period of the disease has 
passed, massage and electricity should be applied to the 
paralyzed parts, but let the brain severelv alone. The 
effect of electricity upon the circulation within the skull is 
altogether too uncertain to justify us in tampering with it in 
any way. Electricity applied to the extremities can do no 



552 THE NERVOUS DISEASES OF CHILDREN. 

harm, and serves an excellent purpose as a means of exer- 
cising parts that cannot be moved by the will. As these 
parts respond to the faradic as well as to the galvanic cur- 
rent, I am in the habit of using the former chiefly, and of 
using a current strong enough to produce mild contrac- 
tions. Powerful currents should be avoided. The electric- 
ity should be administered in sittings from ten to fifteen 
minutes every day, or every other day, and should be given 
by the physician, or by an extremely competent nurse 
under the guidance of the physician. If passive movements 
are combined with ordinary friction the paralyzed parts will 
be kept in a good state of nutrition, and the tendency to 
contracture may be overcome. 

As in the cases of spinal paralysis, so in these cerebral palsies, orthopedic 
measures should be resorted to as soon as contractures have become perma- 
nently established. I have seen many a child walk about fairly well a few 
weeks after the orthopedic surgeon has been allowed to cut the tendons, and 
to provide the proper orthopedic appliances. Contractures, if once formed, 
are rarely, if ever, recovered from in spontaneous fashion, and if it can be 
proved that the contractures are the chief hindrance to the child's progress in 
walking, full liberty should be given to the orthopedic surgeon ; or the phy- 
sician himself, if competent, should apply the well-known orthopedic meas- 
ures. I have been especially pleased with what Dr. Gibney has been able 
to do in several cases of marked disturbances of the upper extremities that 
have occurred in cases of infantile cerebral palsy. I refer in particular to the 
persistent athetoid-choreic movements of the upper extremities so common 
in these cases. By the application of some simple form of restraint such 
post-paralytic movements may be successfully inhibited. In the case of a 
young man, seventeen years of age, who had acquired a cerebral palsy when 
he was ten months of age, and who had exhibited very annoying athetoid 
movements of the upper extremities from that time on, the simple splint 
arrangement which prevented the possibility of these movements, and which 
was worn for a number of months, was sufficient to inhibit the athetoid 
movements altogether ; and in still another case a marked post-hemiplegic 
chorea was much improved, if not altogether inhibited, by placing the arm in 
a plaster-of-Paris bandage for a period of two months. 

The treatment of the idiocy must be conducted on the same lines as in 
other forms of idiocy (see Chapter XXXIII). 

By far the most important task is the treatment of the 
epilepsy associated with the palsy. We have stated else- 
where that this epilepsy is due to the secondary changes in 
the brain, which have developed as a natural result of the 



INFANTILE CEREBRAL PALSIES. 553 

original lesion which gave rise to the palsy. In all these 
cases the epilepsy is practically a focal epilepsy, although 
its clinical manifestations are not often of the strictly Jack- 
sonian order. It was quite natural that Horsley's sugges- 
tions with regard to the proper treatment of focal epilepsy 
should be applied to these forms of epilepsy with infantile 
palsies. 

Horsley himself has reported upon two such patients, and in both of these 
a cystic condition was found. Freud reports a case of Oppenheim's in which 
a meningeal cyst over an arm centre was evacuated, but the brain itself was 
not operated on by the surgeon ; the athetosis and epilepsy in these subjects 
were considerably diminished. The author has had several cases of cerebral 
palsy with epilepsy operated upon. The first case, a boy, six years of age, had 
acquired right hemiplegia at the age of five, and soon thereafter developed 
convulsions, which invariably began in the right hand. This right hand also 
exhibited athetoid movements. Dr. Gerster trephined over the motor centre 
for the right arm, and exposed the greater part of the arm and leg centre in 
the left half of the brain. The boy did well for three months after the op- 
eration ; his attacks disappeared altogether, but the ultimate result cannot be 
stated, as I lost sight of the patient. 

Another case was that of a girl, sixteen years of age, with right hemiplegia 
and epilepsy. Wyeth performed the first operation on this patient, exposing 
the dura over the entire motor area, and a very large portion of the cortex 
was inspected after the dura had been incised. The exposed cortical tissue 
was tested by the faradic current, and responded normally to mild currents ; 
as the cortex seemed to be normal I did not urge its excision. I regretted at 
that time not having urged the complete excision of the arm centre. I do 
not, however, consider Horsley's reason for removing such centres entirely 
valid, since secondary changes, which have been established, seem, after all, to 
be the main source of irritation, and if one focus of disease be removed I am 
confident that other parts of the hemispheres will soon attain to the dignity 
of epileptogenous centres. It is, furthermore, well established at the present 
day that convulsive seizures may result from irritation of almost any part of 
the cortex. In this case there was very little improvement excepting that the 
epileptic attacks were transferred after the operation to the other half of the 
body, whose centres had not yet been exposed. I have had the other half 
of the brain exposed more recently by Dr. Gerster, but for reasons which I 
have suggested did not urge the excision of any of these parts, and I fear 
that the girl will remain an incurable epileptic. 

A third case was one operated on by Dr. Wyeth. The patient was a 
man, thirty-two years of age, who had had an apopletic attack at the age of 
two years. As a result of this he exhibited a left hemiplegia with considerable 
atrophy, with contractures of the left upper extremities and athetoid move- 
ments of the left hand ; furthermore, pes equinus of the left side with a 



554 THE NERVOUS DISEASES OF CHILDREN. 

tendency to equino varus. I made the diagnosis of an old infantile spastic 
hemiplegia, probably due to meningeal hemorrhage over the arm centre in 
the right hemisphere. Dr. Wyeth exposed this centre according to my di- 
rections, and at this point was found a distinct hemorrhagic discoloration of 
the pia, with the development of adhesions between the pia and the cortex. 
I advised that these adhesions be incised, but did not urge removal of the 
cortical tissue, though in this case I am free to say, since the arm was al- 
ready paralyzed and practically useless, a very liberal excision of this arm 
centre might well have been practised. The man recovered well from the 
operation, and his convulsive seizures have been very much diminished dur- 
ing a period of nearly three years. In October, 1894, Dr. Gerster operated 
upon a fourth case (infantile hemiplegia with epilepsy) in a boy, aged twelve ; 
the epileptic lit began in the left (paralyzed) arm, and then became general. 
The greater portion of the arm centre was excised, showing the effects of old 
hemorrhage (Fig. 145) ; the attacks have not been -sensibly diminished. Sim- 
ilar cases have been reported by Weir, Keen, Roswell Park, Angell, and Starr. 
In Weir's case a large cyst was found, which was carefully drained, but the 
child, four years of age, died five hours after the operation. In one of Keen's 
cases the child died twenty minutes after the operation, and the brain exhib- 
ited a very considerable atrophy of the entire left hemisphere. The greater 
danger of all these operations in young children should be taken into ac- 
count, and for this reason I now hesitate to have the operation performed 
upon any child under the age of four years. Wildermuth has reported two 
cases. In the one case he removed atrophic cortical tissue, and evacuated a 
cyst underlying it. Three years after the operation the patient was said to 
have been improved. In another case he also performed excision of an 
atrophic portion of the cortex, and the attacks were said to have been in- 
hibited up to ten months after the operation. 

Dr. Angell, of Rochester, has reported upon a case of infantile hemiple- 
gia (birth case) with imbecility and epilepsy. A simple craniotomy was 
done, exposing a cyst in front of and above the ascending frontal region of the 
left side ; a slight improvement followed. If a cyst is as clearly limited as in 
this case, removal of the cyst, or at least drainage of the same, would have 
been a more rational and radical operation ; the improvement following upon 
craniotomy can be explained as the result of a release of pressure. 

These results have not been such as to create any great surgical enthu- 
siasm, though if all the symptoms of a case point clearly to an initial focus of 
disease, and if the epilepsy cannot be inhibited by any other measures, sur- 
gical procedures are quite in order. As cysts are extremely frequent in these 
infantile cerebral palsies, the evacuation and removal of such cysts will often 
be followed by considerable relief. I believe that it is due to the frequency 
of such cysts that relief follows so many operations for epilepsy, and that it 
is not the mere effect of the operation per se, as White would have us be- 
lieve. 

If the epilepsy does not yield to surgical measures, or 
if surgical procedures cannot be practised, the ordinary 



INFANTILE CEREBRAL PALSIES. 555 

anti-epileptic measures may be employed. Bromides and 
chloral should be given in moderate doses, but there is little 
use in pushing these drugs to the extreme, for we cannot ex- 
pect to cure an epilepsy that is dependent upon an organic 
brain lesion. 

BIBLIOGRAPHY. 

(The extensive literature of this subject will be found in the monographs 
of Freud-Rie, and in the articles by the author and Peterson.) 

Bernhardt : Virchow's Arch., vol. cii., 1885. 

Benedikt, M. : Elektrotherapie. Vienna, 1868. 

Bourneville : Recherches cliniques, etc. Paris, 1890, 1891, 1892. 

Cotard : JEtude sur l'Atrophie partielle du Cerveau. Paris, 1868. 

Dejerine : Archives de Physiologie, vol. xxiii., 1891. 

Freud und Rie : Klinische Studie liber die halbseitige Cerebrallahmung der 

Kinder. Vienna, 1891. 
Freud, S. : Cerebrale Diplegien. Vienna, 1892. (Full literature to be found 

in this and the preceding monograph.) 
Gowers : Diseases of the Nervous System. Am. Ed., vol. ii. 
Hadden : Infantile Spasmodic Paralysis, Brain, vol. vi., 1884. 
Heine, J. Von : Spinale Kinderlahmung, i860. 

Henoch : Lectures on Children's Diseases, New Sydenham Soc. Pub., 1889. 
Jendrassik et Marie : Arch, de Phys., vol. v., 1885. 
Kast: Arch. f. Psych., vol. xviii., 1887. 

Knapp, P. C. : Journal of Nervous and Mental Disease, 1887, p. 480. 
Kundrat : Die Porencephalic Vienna, 1882. Wiener kl. Wochenschr., 1890. 
Little: Obstetr. Soc. Trans., 1862, vol. iii. ; Treatise on Deformities, 1853; 

also in Holmes's System of Surgery, 1870. 
Lovett : Boston Medical and Surgical Journal, June 28, 1888. 
McNutt, Sarah : American Journal of the Medical Sciences, January, 1885 ; 

American Journal of Obstetrics, January, 1885. 
Osier : The Cerebral Palsies of Children, 1889; Medical News, Philadelphia,. 

1888. 
Peterson : Article in L. Starr's Text-book of Diseases of Children, 
v. Recklinghausen : Pathologie des Kreislaufes und der Ernahrung, 1SS3. 
Sachs and Peterson : Journal of Nervous and Mental Disease, May, 1890. 
Sachs, B. : Journal of Nervous and Mental Disease, 1887 ; New York Medical 

Journal, May, 1891 ; or in Keating's Cyclopaedia, vol. iv. ; Volkmann's 

Sammlung, Nos. 46, 47, 1892 ; Journal of Nervous and Mental Disease, 

August, 1892. 
Stnimpell: Jahrb. f. Kinderheilk., N. F., 1884, vol. xxii. ; Deutsche Med. 

Wochenschr., October 17, 1889; Deutsches Arch. f. kl. Med., vol. xlvii. 
Wallenberg : Jahrb. f. Kinderheilk., vol. xxiw, 1886. 

Willard and Lloyd : American Journal of the Medical Sciences. April. 1892. 
Wuillaumier : These de Paris, 1882. 



CHAPTER XXIX. 

TUMORS OF THE BRAIN AND ITS MENINGES. 

New-growths within the skull are very common in 
childhood. There is no period of early life that is entirely 
exempt from them. Careful studies of cerebral tumors 
in children have been made by Steffen, Bernhardt, Starr, 
Knapp, and others. The following tables are taken from an 
article by Peterson : 

Table I. 

Form of Tumor. No. of Cases. 

Tubercle 166 

Glioma 42 

Sarcoma 37 

c y st 35 

Carcinoma 11 

Gliosarcoma 5 

Angiosarcoma 1 

Myxosarcoma „ 1 

Papillary epithelioma 1 

Gumma 1 

Not stated 35 

Total • . 335 

Table II. 

Site of Tumor. No. of Cases. 

Cerebellum 105 

Pons Varolii 42 

Centrum ovale 41 

Basal ganglia and lateral ventricles 30 

Corpora quadrigemina and crura cerebri 25 

Cortex cerebri 23 

Medulla oblongata 7 

Fourth ventricle 6 

Base of brain 8 

Total 287 






TUMORS OF THE BRAIN AND ITS MENINGES. 557 

From these tables we can infer, first of all, that tubercle is much more 
frequent than any other form of intra-cranial neoplasm, very nearly one-half 
of all the cases being of this character. Glioma and sarcoma are the next 
most common forms, and as the cerebral tissue in earlier years is subject to 
proliferation, the frequent occurrence of the former need not be a matter of 
surprise. Cysts differ from tumor in this respect that they are secondary 
products of some preceding morbid process, very often resulting from an old 
hemorrhage, or from embolic softening. The contents of a cyst may remain 
stationary for a very long time, and under such circumstances the symptoms 
will not be very like those of a growing tumor. In other instances the con- 
tents of the cyst suddenly increase and all the symptoms may be those of a 
rapidly increasing neoplasm. 

As for the location of tumors in general, it will be seen that a larger 
number are found in the cerebellum than in any other part of the brain. 
The pons and the centrum ovale are next in order of frequency, then come 
the basal ganglia, and last the cortex of the brain, and the region of the cor- 
pora quadrigemina. The medulla oblongata and the base of the brain are 
rarely the seat of tumor. 

Etiology. — The actual cause of cerebral tumors in 
children is a matter of considerable speculation. It is only 
with regard to gliomata that we can state that the natural 
tendency of the infantile brain to proliferation of its tissue 
upon the slightest provocation accounts for the frequent 
occurrence of this special form of neoplasm. Tubercle, 
sarcoma, and carcinoma of the brain, are always secondary 
to deposits of a similar character in other parts of the body. 
Carcinoma is rare in children, and carcinomatous deposits 
in the brain are correspondingly infrequent, but under excep- 
tional conditions these may occur early in life. A few cases 
have been observed which were due to an invasion of the 
growth from the orbit. The exact relation of traumatic in- 
juries to new-growths has been often in dispute. It is more 
than probable that the accident to which the tumor is re- 
ferred is, as a rule, merely a landmark in the child's 
history. 

In the fewest instances only can an actual causal relation between the two 
be established. The hyperasmic condition or the slight hemorrhages which 
occur at the seat of injury may at times be sufficient to become the starting- 
point of a latent morbid process. Boys are more liable to brain tumors than 
girls, and those who are not too careful in utilizing statistical evidence will 
claim that this fact is to be explained by the greater frequency of injuries to 
the boys' skulls. 



558 THE NERVOUS DISEASES OF CHILDREN. 

Symptoms. — As in the adult, so in the child, the symp- 
toms of intra-cranial tumor may be divided into two distinct 
groups. The first includes the general symptoms resulting 
from increased pressure, and the second group includes 
those due entirely to the location of the tumor. It is a 
matter of course that slowly progressive tumors will exhib- 
it less symptoms than those which invade the brain rapidly 
and soon attain to a very considerable size. A tumor of 
small size growing rapidly, will produce far more symp- 
toms than one of huge size which has taken years to de- 
velop. The symptoms will also vary according to the 
blood-supply of the tumor, and according to the increase 
in the amount of hydrocephalic fluid. The general symp- 
toms which we must consider somewhat in detail are head- 
ache, nausea, vomiting, insomnia, convulsions, and double 
optic neuritis. 

Headache is in many cases the most striking symptom of tumor of the 
brain. The nearer the tumor is to the cortex, and the more it involves the 
meninges, the more intense on the whole the headaches are likely to be ; but 
I have seen headaches as intense as those of cortical tumors in cases in which 
the growth was in the vicinity of the ganglia, or even at the base of the brain. 
In such patients the increase in cerebro-spinal fluid has much to do, in all 
probability, with the development of the headaches. The intense " boring," 
" gnawing " pain is generally referred to that part of the head which is nearest 
to the new-growth ; but there are exceptions to this rule as to every other. 
In children whose fontanelles are not completely closed the tumors may 
attain to a considerable size without causing intense headaches. These 
headaches are often the cause of sleeplessness, and both the pain. and the 
insomnia are largely responsible for the rapid emaciation of the child. In 
some few instances insomnia is present without pain, and is the result of 
cerebral excitement. 

The association of nausea and vomiting with headaches in a child should 
lead to the suspicion of tumor, particularly if all these symptoms persist for 
some considerable period of time. The vomiting is characterized by its sud- 
denness, and by its projectile character. Nausea may be associated with it, 
but in many cases there is no warning given of the vomiting, and it comes as 
a surprise upon the child, and often occurs entirely independently of meals ; 
at other times during meals. Vertigo is not infrequent in cases of tumor, and 
is very apt to occur with every change in position of the head. It is very fre- 
quent indeed in connection with tumors of the cerebellum, or of the pons, 
which is explained by the relation of these parts to the auditory nerve. 

Convulsions in children are so common that they alone need not give rise 
to the suspicion of tumor, but if associated with constant headaches, with 



TUMORS OF THE BRA IN AND ITS MENINGES. 559 

vomiting, and if all these symptoms are frequently repeated, the suspicion may 
be well founded. These convulsions are very often general, but in addition 
to the general convulsions, or without the occurrence of such, localized con- 
vulsions may occur if the neoplasm is in some part of the motor area. Such 
localized convulsions, associated with headaches, vomiting, and with optic 
neuritis, point very definitely to an intra-cranial growth. 

Double optic neuritis and the complete optic atrophy 
following it are important symptoms of intra-cranial tu- 
mor. It is certain that there is no symptom which if pres- 
ent, is more valuable than an optic neuritis ; but, on the 
other hand, tumors may attain considerable size without 
the development of optic neuritis. This neuritis is in all 
probability due to increase of intra-cranial pressure. Other 
theories which have been proposed do not explain all the 
peculiarities of optic neuritis as well as the theory of in- 
creased pressure does. The fundus should be examined in 
every doubtful case of brain tumor, for normal vision is 
not incompatible with a considerable degree of optic neu- 
ritis, and changes in the optic nerve may occur, and do oc- 
cur, with tumor in any part of the brain ; but they occur at 
a much earlier period and to a much more intense degree 
with tumors situated at the base of the brain. Under these 
conditions the direct pressure upon the optic chiasm and 
the greater increase of intra-cranial fluid at the base may 
account for the earlier appearance of the symptoms. The 
optic neuritis is generally double, though it may be more 
distinct for a time in one eye than in another. 

Sudden variations in the pulse-rate, and in rapidity of res- 
piration have been observed ; Cheyne-Stokes respiration I 
have seen in children suffering from cerebellar tumors long 
before the terminal stage had been reached. 

The presence of ,tumor can often be suspected from the 
occurrence of these general symptoms. The signal or 
localizing symptoms are dependent upon the special func- 
tions of the areas invaded by the neoplasm. These localiz- 
ing symptoms are the best illustrations in man of cerebral 
functions, as they have been demonstrated by experiments 
upon animals. The matter is not quite as simple, however, 
as it would seem, for a neoplasm causes both direct and in- 
direct symptoms. The direct symptoms are those caused 



560 THE NERVOUS DISEASES OF CHILDREN. 

by the actual loss of function due to displacement of tissue 
by the neoplasm. The indirect symptoms are caused either 
by the distant effects of the pressure by the tumor upon 
neighboring parts, or the)' may result from the secondary 
increase of intra-cranial fluid. At all events, the symptoms 
first produced are the most important ones, and will be of 
the greatest value in determining the exact site of the 
lesion. 

The indirect symptoms are perhaps more frequent in the cases of cere- 
bellar tumors than in any other. Thus in this form we may have the 
general symptoms of intra-cranial neoplasm, together with reeling and dis- 
tinct occipital headaches. Associated with these we often find a paralysis 
of the rectus externus supplied by the sixth nerve. While this sixth-nerve 
paralysis is an indirect symptom of cerebellar tumor caused by pressure of 
the nerve lying between the base of the brain and the skull, it is practically 
due to a squeezing and stretching of the nerve in consequence of the push- 
ing forward of the tentorium. 

Macewen's Symptom. — " The elicitation of a differential cranial per- 
cussion-note as an aid to cerebral diagnosis in certain gross changes of the 
intra-cranial contents, especially in children," has been practised by Macewen 
for many years and described in his monograph on " Infective Diseases of the 
Brain and Spinal Cord." For the present, the symptom is most valuable in 
the case of abscesses and of over-distended lateral ventricles. In cases of 
tumor near the surface of the brain the percussion-note will prove of service. 
I have noted a dulness in several cases of tumor of the brain (one case in 
the adult and two in children) ; if the lateral ventricles are over-distended 
the resonance is greatly increased. But distention of the ventricles may be 
secondary to tumor, and in this way increased resonance of the percussion- 
note may be significant. The percussion-note of normal crania seems to vary 
much ; it will be best, therefore, to use this symptom very cautiously. The 
note can be elicited best by tapping the skull lightly with the finger or 
percussion-hammer, and receiving the note through a stethoscope placed 
upon the pterion, or upon the middle of the forehead. 

Tumors of the Cortex.— It is not an easy matter to distinguish be- 
tween the purely cortical tumors and those situated in the subjacent white 
matter. Some claim that it is impossible to differentiate between cortical 
and subcortical tumors, but this seems to me to be rather overstating the 
facts. While the localizing symptoms may be exactly the same in both 
these classes of tumors, the order of development of the symptoms will 
be different, and may give some indication of the exact site of the tumor. 
In subcortical tumors the march of the symptoms is generally such that 
the approach of the lesion toward the cortex is marked by a special series 
of symptoms. Taking, for example, the tumors in the motor area, those 
in or near the gray matter, however small, are apt to give rise to occa- 



TUMORS OF THE BRAIN AND ITS MENINGES. 56 1 

sional convulsive seizures from the very start ; whereas, in the tumors which 
begin in the subjacent white matter, the paralytic symptoms may appear at 
a very early day and may exist for some time before any symptoms of corti- 
cal irritation arise. Thus, in a case under my observation, the patient had 
gradually developed paralysis of the arm, of the face, and slight paresis of 
the leg on one side of the body. He had indistinct headaches and optic 
neuritis, and yet, during the first three months after the onset of these symp- 
toms, not a single convulsive twitching occurred. Since that period epilep- 
toid seizures have set in, and the occurrence has proved that the tumor has 
finally invaded the cortical region. The invasion of the cortex, moreover, is 
more likely to lead to repeated convulsive seizures and to increased head- 
aches from the more direct involvement of the meningeal coverings ; the 
nearer the surface and the nearer the meninges the more intense these head- 
aches become.* Certain classes of tumors, moreover, are more apt to begin 
in the cortical tissue than in the subcortical white substance ; thus tubercles, 
gliomata, and gummata, above all, are almost certain to begin near the sur- 
face, while sarcomata and cysts are as often subcortical as cortical in origin. 

Tumors of the Frontal Lobe. — Since the frontal lobe has no dis- 
tinct and special function, tumors in this region are often developed without 
localizing symptoms. If the tumor extends downward and involves the ol- 
factory bulb the sense of smell may be diminished or lost ; but changes in 
character and intelligence, irritability or stupidity, are now considered on all 
sides to be relatively frequent in diseases of the frontal lobe, and this is en- 
tirely in support of the conditions which Goltz first discovered as a change in 
the behavior of dogs whose frontal.lobes were excised or destroyed. More 
recently Torje has recorded a case in which there was marked disturbance of 
salivation ; he suggests that the drooling in idiots may be due to defective 
frontal development. Further observations will be needed to confirm this 
view. A suspicion of frontal tumor may also be entertained if the general 
symptoms were present for a long period of time, and then symptoms point- 
ing to the third frontal gyrus or the motor areas appear as evidence of 
the gradual encroachment of the new-growth upon these areas. 

Tumors of the Third Frontal Convolution.— The third frontal 
convolution is the seat of motor speech function, and any destructive lesion 
in or near this part of the brain will cause typical motor aphasia. Agraphia 
may be associated with motor aphasia. The cases in which motor aphasia, 
or agraphia, or both, have signalized the invasion of a tumor are compara- 
tively rare, particularly in children ; for if the invasion of the tumor be a 
gradual one the right hemisphere may, in right-handed persons, gradually as- 
sume the functions of speech, which up to that time have been inherent in the 
left hemisphere. In addition to this, in young children the differentiation 
between the left and the right hemispheres is not nearly as complete as in 
later years. 

Tumors of the Motor Area. — These can be recognized more readily 

* If there is excessive intra-cranial pressure, headaches may be intense, even in 
tumors distant from the surface. 



562 THE NERVOUS DISEASES OF CHILDREN. 

than those in any other part of the cortex. In the earlier stages of the dis- 
ease symptoms of cortical irritation are present, and convulsive twitchings of 

the paretic or paralyzed part will indicate the probable site of the tumor. 
Thus, if a patient with slight sensory and motor disturbances in the upper 
extremity should experience occasional convulsive twitchings of the arm, of 
the forearm, or even of the thumb alone, the character and extent of these 
twitchings would point to the part of the brain first and chiefly involved. 
According to the course and development of the convulsive seizures the di- 
rection in which the growth extends can be judged. A reference to Fig. 
122 will suffice to enumerate the symptoms to be expected in any case. 

Tumors of the Parietal Lobe. — Tumors of the parietal lobe may 
cause no distinct localizing symptoms, but the records of Dana and others 
prove conclusively that lesions in these parts are apt to be followed by sen- 
sory changes in the limbs of the opposite half of the body. These facts of 
human pathology are in keeping with the results of Munk's experiments on 
dogs, and those of Schafer and Horsley on monkeys. These sensor}' symp- 
toms, however, are not so constant that their absence would militate against 
the diagnosis of tumor in this region. If a tumor in the parietal lobe affects 
the subjacent white matter it may result in hemianopsia, for the white tract 
of Gratiolet passing from the internal capsule to the occipital lobe lies under 
this portion of the cortex. Ferrier's claim, that the centre of vision lies in 
the angular gyrus, is due to the involvement by disease in this region of the 
optic radiations which we have mentioned. By extension to the inferior 
parietal lobule the tumor may give rise to that peculiar disturbance of speech 
known as word-blindness. The patient cannot read and write at will, though 
he may be able to write upon dictation, or to copy written signs. In chil- 
dren, excepting those of a more advanced age, cases will rarely arise in which 
word-blindness or word-deafness are important symptoms. According to 
Wernicke the conjugate movements of the eyes are governed by a centre in 
the inferior parietal lobule. If there is distinct impairment of this one func- 
tion, disease in this region may therefore be suspected. 

Tumors of the Occipital Lobe.— Tumors in the occipital lobe are 
recognized by the peculiar disturbances of vision. As each occipital lobe is 
connected with one-half of each eye. tumor in this region is signalized bv 
homonymous hemianopsia without any other special symptoms ; convulsions 
may occur, and not necessarily on account of the direct extension of the le- 
sion into the motor areas, but because, as has been shown by Binswanger. 
disease in any part of the cortex may cause epileptic disturbances. The re- 
searches of Nothnagel and Seguin, which have been corroborated by other 
authors, point with a great degree of certainty to the cuneus as the actual 
centre of vision. We must, therefore, consider the possibility of a tumor be- 
ginning on the median surface of the occipital lobe and gradually extending 
to the outer surface. 

Tumors of the Temporo-sphexoidal Lobe.— The temporo-sphe- 
noidal lobe contains the centres for hearing and for sensory speech ; it is, 
therefore, natural to expect that in the case of tumor in these auditor}- re- 
gions hearing will be impaired, not abolished, on the side opposite the lesion, 



TUMORS OF THE BRAIN AND ITS MENINGES. 563 

and sensory aphasia will be present. The patient will be able to speak spon- 
taneously and correctly, but will not be able to understand what is being 
said to him, and will, of course, not be able to repeat spoken language. The 
differential diagnosis between tumor and abscess (so frequent in this region) 
should be established carefully. 

Tumors of the Basal Ganglia. — Tumors of the basal ganglia and the 
adjacent parts are not rare. The symptoms which they produce are, as far 
as we know, entirely due to direct or indirect involvement of the internal cap- 
sule. As this capsule contains the entire motor tract for the opposite side of 
the body, the sensory fibres, the fibres of special sense, the speech-tracts, we 
can infer the multiplicity of symptoms which may result from such a le- 
sion. The position of these fibres in the internal capsule is quite well es- 
tablished, and in the case of small tumors, the order of involvement of the 
different functions may give some clew as to the direction in which the tumor 
is extending. The tumor may impinge, too, upon the lateral ventricles, and 
thus cause considerable disturbance. Some of the symptoms may be sec- 
ondary to the increase of hydrocephalic fluid. On account of the compact 
structure of the inner part of the brain, even small tumors will cause con- 
siderable distortion of the parts, and in addition to all the other symptoms 
presented as the result of the involvement of the internal capsule, we may 
have symptoms resulting indirectly from pressure upon the cranial nerves, 
which come off from the brain axis below this region. The differential diagno- 
sis between tumors of the ganglia and tumors of the cortex will be based in 
part upon the absence of convulsive seizures in the case of ganglionic tu- 
mors, and the more frequent presence of choreic or athetoid movements 
with cortical disease. 

Tumors of the Crus Cerebri. — A neoplasm in this region can be 
recognized very early by the association of oculo-motor symptoms with paral- 
ysis of motion and sensation of the opposite half of the body. The eye 
will exhibit ptosis, paralysis of all the external muscles except the rectus ex- 
ternus and the superior oblique, and complete paralysis of the sphincter 
iridis, and the ciliary muscle. 

Both peduncles are so close to one another that a tumor occurring in one 
may actually invade the opposite side, or at least press against the peduncle 
of the other half of the brain. For this reason it is not uncommon to find 
paralysis of both halves of the body, or possibly double ptosis and double 
oculo-motor symptoms in tumors in this region. In the majority of cases. 
however, the symptoms are strictly unilateral for a long period, and become 
bilateral later on. 

Tumors of the Corpora Quadrigemina. — These tumors should 
in reality be considered with tumors of the peduncles, for these structures are 
so closely related to one another, anatomically and physiologically, that the 
occurrence of neoplasm in the one part or the other will produce a common 
series of symptoms. It is only the occurrence of oculo-motor paralysis with 
opposite hemipleglia that suggests the region of the peduncles, while a few 
additional symptoms refer distinctly to the corpora quadrigemina. These 
additional symptoms are due to the relation which the corpora quadrigemina 



564 THE NERVOUS DISEASES OF CHILDREN. 

bears to visual functions, and to the connection between the former and 
the cerebellum. Loss of pupillary reflexes, nystagmus, vertigo, and a 
condition resembling cerebellar ataxia, point to the region of the corpora 
quadrigemina as the special seat of the lesion. I had occasion a few years 
ago to publish an interesting case of this description, with the result of the 
post-mortem examination. 

A child, three years of age, was first examined by Doctor Meeks in Nov- 
ember, 1889. At that time it presented a double ptosis, but no other ocular 
paralysis ; it was dull and listless, and its pulse ranged between 160 and 165 ; 
the temperature was normal. It would sit quietly all day long ; if it at- 
tempted to walk, it w T ould stagger and fall. There was no anaesthesia or ataxia, 
and the knee-jerks were absent. There was ptosis of both eyelids, the pupils 
being half covered, no other paralysis of ocular muscles, no nystagmus ; 
there was at this time a suspicion of optic neuritis. In December, 1889, the 
pulse was 140, and the temperature in the axilla 100. 2 F. ; there was double 
and almost complete ptosis ; there was no upward or downward movement of 
either eye ; both internal recti muscles were thrown into a condition of clonic 
spasm when an attempt was made to use them. Both internal recti were 
capable of very slight movements, and all other external ocular muscles were 
completely paralyzed. There was some reflex contraction during accommo- 
dation, and also slight contractility to light in both pupils. There was also 
slight paresis of the left half of the face. Vision was very much impaired. 
The child was in a condition of semi-stupor, yet was able to walk a little, and 
exhibited in doing so a most distinct cerebellar walk, with a tendency to fall 
to the right side. The reflexes were exaggerated in the upper, as well as in 
the lower extremities, and there were distinct occipital headaches. The 
child was able to use all four extremities, although those on the right side 
were weaker than those on the left. The diagnosis of tumor of the corpora 
quadrigemina was made, and this tumor was supposed to be associated with 
a general tubercular affection. The child grew rapidly worse, had convulsive 
seizures, became blind, comatose, and two weeks before death developed left 
hemiplegia. The child died February 4, 1890, four months after the onset of 
the symptoms. 

At the autopsy a solitary tubercle was discovered, near the right lateral 
sinus, about one centimetre in diameter, and pressing against the lateral 
edges of the cerebellum, producing thrombosis of the lateral sinus. Other 
tubercles surrounded by large areas of softened tissue were found in the 
cerebellum. There were small tubercular deposits along the blood-vessels 
in the hemispheres, but with this exception the hemispheres were entirely 
normal. There was unusual thickening of the pia, w 7 ith small tubercular de- 
posits between the corpora mammillaria and the optic chiasm, and in the 
interpeduncular spaces the thickening of the pia was extremely marked, but 
of very recent date. In consequence of this thickening, the third and sixth 
nerves were twisted out of their normal position. These conditions evidently 
gave rise to the rapidly increasing symptoms of later weeks, but the earlier 
symptoms of the case and those which alone attracted attention for a long 
while, were explained by the tumor found in the region of the corpora qua- 



TUMORS OF THE- BRAIN AND ITS MENINGES. 



565 



drigemina, as will be seen from the annexed Figure 148. The tumor was a 
solitary tubercle of very considerable size ; it occupied chiefly the tegmental 
portion of the crus, and almost completely occluded the aqueduct of Sylvius. 

The symptoms in this case are in entire keeping with the conclusions of 
Nothnagel, Christ, and others, and from the conclusions of these authors, as 
well as from my own former studies, we may infer that the slow onset of the 
oculo-motor symptoms, with cerebellar ataxia, with vomiting and optic neu- 
ritis, point to a neoplasm in the vicinity of the corpora quadrigemina. 

Tumors of the Pons. — Tumors of the pons and medulla, like other le- 
sions in these parts, give rise to a great multiplicity of symptoms. The pyram- 
idal tracts are closely approx- 
imated to one another near the ^m**-' v % 
median line, so that, though the 
symptoms may be unilateral, 
they are often bilateral. In ad- 
dition, therefore, to the hemiple- 
gia, or double hemiplegia, we 
may have other symptoms point- 
ing to an involvement of the 
various cranial nerves, A neo- 
plasm in the upper half of the 
pons may encroach more upon 
the peduncles than upon the 
structures of the pons itself, re- 
sulting in a hemiplegia of one 
side of the body with an in- 
volvement of the third and fifth 
nerves of the opposite side. If 
the tumor is in the lower half 
of the pons, the fifth, sixth, sev- 
enth, and eighth nerves will be 
more or less involved, and the 
symptoms resulting from this 
affection will be paralysis of the 
rectus externus, paralysis of all 
the branches of the seventh nerve 
in one half of the face, and a loss 

of hearing in one ear. All these cranial nerve symptoms will be on the side of 
the lesion opposite the hemiplegia. If the sixth nerve nucleus is affected there 
will be, in addition, distinct paralysis of one rectus externus muscle, and pa- 
ralysis of the conjugated movements of the eyes toward the side of the lesion, 
for this nucleus is connected with the third nerve nucleus of the opposite side 
and governs the outward movement of each eye. In spite of this conjugate 
paralysis each internal rectus if examined separately may exhibit normal 
movements. If the lesion is near the surface of the pons, and away from the 
nucleus, it will involve the root of the sixth nerve, and will cause paralysis of 
the rectus externus muscle of one side, but will not affect the conjugate 




Fig. 148. — Section passing through the Pos- 
terior Quadrigeminal Bodies. T, tumor (sol- 
itary tubercle) ; P. Q, posterior quadrigemi- 
nal body. 



566 THE NERVOUS DISEASES OF CHILDREN. 

movements of the opposite side. If the patient is directed to look toward 
the side of the paralyzed rectus or toward the side of the tumor the opposite 
eye will move promptly, the affected eye remaining fixed. This differentia- 
tion between the isolated paralysis of the rectus externus and paralysis of the 
conjugated movements is the most valuable, and perhaps the only differen- 
tial point of diagnosis that helps at times to distinguish between a tumor 
near the surface and one within the substance of the pons. 

The difficulties of diagnosis of pons lesions are increased by the exceeding 
variability of the symptoms, and by the fact that some of the nerves in the 
pons escape disease, whereas others may be intensely affected. 

In the case of a tumor in the medulla oblongata the symptoms are very 
similar to those met with in bulbar palsy. The symptoms will indicate dis- 
ease of the glossopharyngeal, of the vagus, of the spinal accessory, and of 
the hypoglossal nerves, together with a unilateral or bilateral paralysis of the 
arms or legs ; the facial nerve will not be involved. Difficulties in degluti- 
tion, in respiratory and cardiac movements ; paralysis or spasm of the sterno- 
cleido mastoid, and of the trapezius ; paralysis and atrophy of the tongue, 
together with vomiting, with glycosuria or polyuria — all these signs will 
suggest a lesion in the medulla. Tumors in this region, and particularly 
gummata, are not rare. Difficulties in diagnosis often arise on account of 
the bilateral character of all the symptoms, but it should be remembered that 
the two halves of the brain at this level are scarcely separated from one an- 
other, and that it is natural for all but vascular lesions (and even these may 
do so) to produce bilateral symptoms. In the pons and medulla the sensory 
tracts also lie very closely to the motor, and for this reason the symptoms 
may be still further complicated by the occurrence of partial, or complete, or 
double hemiansesthesia. As the cerebellum is also in close juxtaposition to 
the medulla and the pons, symptoms of cerebellar involvement may be pres- 
ent in the cases of neoplasms, both in the pons and in the medulla. Optic 
neuritis is developed at a very early day, and occipital headaches are par- 
ticularly severe. 

Tumors of the Cerebellum. — Tumors of the cerebellum are the 
most frequent. The first symptoms are occipital headache, projectile vom- 
iting, and (at a relatively early period) a peculiar staggering or reeling gait 
(cerebellar titubation). This resembles very much the staggering of a drunken 
man ; it is often so severe that the person falls over in a heap to one side 
or the other if he is not properly supported. This movement is supposed to 
be due to involvement of the middle peduncle. Vertigo is also more fre- 
quent and more severe in cerebellar tumors than in many others. The cere- 
bellum being separated from the rest of the brain by a very rigid membrane 
(tentorium), every increase in its size naturally produces serious symptoms 
resulting either from direct pressure or from the increase of cerebro-spinal 
fluid. A cerebellar tumor may be of very small size and yet sufficiently 
large to compress the sixth, seventh, and eighth nerves, and in its further 
growth may produce involvement of the bulbar nerves as well, just as tumor 
of the bulb may also produce cerebellar symptoms. The affection of the 
sixth nerve, causing paralysis of the rectus externus, is extremely common in 



TUMORS OF THE BRAIN AND ITS MENINGES. 



567 



cerebellar tumors, and in some cases the diagnosis of cerebellar tumor may- 
be corroborated by the early involvement of the seventh and eighth nerves. 
In one case of cerebellar tumor in the adult, seen by the author in connection 
with Dr. Stieglitz, the patient complained at first of nothing but dizziness, 
occasional vomiting, slight occipital headaches, and in walking he exhibited 
a very slight degree of staggering, which at times disappeared altogether ; 
but the electrical examinations of the facial nerve of the right side showed 
beginning reaction of degeneration while the paralysis was still very slight, 
and a reaction of degeneration, too, in the auditory nerve, with deafness of 
this same side. Both these symptoms were so slight that they might well 
have been overlooked. As the case progressed the staggering and the facial 
paralysis became more complete, hearing remained permanently involved, 




Fig. 149. — Tumor (Sarcoma) of the Cerebellum. Early appearance of facial and 
auditory nerve symptoms. 



and on the death of the patient the tumor was found in the lateral lobes of 
the cerebellum, immediately impinging upon the roots of the seventh and 
eighth nerves (Fig. 149). 

A single history will illustrate the peculiar features of tumors of the cere- 
bellum in children. This case was that of a boy, nine years of age. The 
mother had nine children, all healthy, no diathesis in any one of the family. 
The boy attributed his trouble to the fact that a little friend once made him 
stand on his head for a prolonged period of time. A few days later he 
noticed that his head inclined to the right side ; three weeks after this he 
began to squint, and had intense headaches, with vomiting. He always re- 
ferred the pain to the left occipital region, which was sensitive to the slightest 
touch. When I saw the child, about four weeks after the first symptoms had 
come on, there was distinct double optic neuritis; both abducens nerves were 
paretic. The reaction to light was very sluggish, but better during accommo- 
dation. There was slight paralysis of the left facial nerve in all its branches; 



568 THE NERVOUS DISEASES OF CHILDREN. 

occasionally, also, he experienced difficulty in opening the mouth ; distinct 
dysarthria, but there was no difficulty in deglutition. The left occipital 
region was painful, and the head slightly inclined to the right side. The 
grasp of the hands was equal, but weak ; the legs were normal, but the knee- 
jerks were not exaggerated. He staggered a little in walking, but there was 
no Romberg symptom. The further progress of the case consisted in in- 
crease of the paralysis of both recti externi, and the left was always more 
paralyzed than the right ; the head was carried more and more toward the 
right side, but he staggered to the left. Vision was good for some time, and 
was not affected up to the very last period. For a few weeks the conditions 
were slightly improved by the inunctions of mercury, but all the symptoms 
returned and gradually increased. After a little while the boy began to lose 
the proper use of his legs, he reeled more and more, so that he could neither 
stand nor walk alone ; both abducens became absolutely paralyzed ; the head 
fell to the right and could not be lifted spontaneously ; vomiting was very 
frequent ; vision became gradually impaired ; the knee-jerks became subnor- 
mal, and the boy gradually wasted away. He died about six months after 
the onset of the symptoms. 

While there is, as a rule, no difficulty in recognizing tumors of the cerebel- 
lum, some of them, particularly if they are of slow growth, produce very few 
symptoms. The general manifestations of cerebellar tumors do not vary 
much from tumors in other parts of the brain ; but if the growth encroaches 
upon the pyramidal tracts in the pons and medulla unilateral or bilateral 
paralysis will follow. The reflexes are sometimes exaggerated, as in all other 
cerebral diseases, but in not a few cases the knee-jerks are either diminished 
or absent. 

Differential Diagnosis. — We must consider the rela- 
tion to abscess, chronic hydrocephalus, tubercular or simple 
meningitis, and cerebral hemorrhage. 

The distinction between tumor of the brain and abscess 
will depend upon the frequent presence of fever, and upon 
the very slow invasion and slow development of all the 
symptoms in the latter condition, also upon the occasional 
absence of optic neuritis in spite of the presence of symp- 
toms pointing to increased intra-cranial pressure ; but the 
differential diagnosis will depend first and foremost upon 
the proof of the presence of such conditions which give 
rise to abscess, or the absence of such conditions in the 
case of tumor. For other symptoms characteristic of 
abscess rather than of tumor, the student is referred to 
the next chapter. 

The ordinary forms of tubercular meningitis are so dis- 
tinct that they cannot be readily confounded with tumor of 



TUMORS OF THE BRAIN AND ITS MENINGES. 569 

the brain, but the difficulty lies in the fact that some cases 
of tubercular affection of the brain take an exceedingly 
slow course, and that some forms of tumor prove rapidly 
fatal. Furthermore, tumor in the form of solitary tubercle 
and meningitis are occasionally associated with one another, 
as was proved in the case referred to among tumors of the 
corpora quadrigemina. In meningitis, whether of a tuber- 
cular character or not, the rapid involvement of a number 
of cranial nerves, without an increase in all the other symp- 
toms, will argue in favor of this disease rather than of 
tumor. This is true also of the specific meningitis as well 
as of the tubercular form. Hemorrhage is ordinarily quite 
distinct from tumor; but apoplectic seizures occur in con- 
nection with various kinds of neoplasm, and may conceal 
the true nature of the morbid process. 

Pathology. — The most important intra-cranial growth 
is the solitary tubercle. It occurs both in children who ex- 
hibit distinct tubercular tendencies, and also in those who 
neither give a family history of tuberculosis nor exhibit 
any cachectic symptoms. It is highly probable that soli- 
tary tubercle is always secondary to tubercular disease 
elsewhere. The mesenteric glands are not infrequently the 
seat of tubercular disease, and may constitute the point of 
origin for the tubercular invasion of the brain or cord. 
But as these are so frequently overlooked in post-mortem 
examinations, the cases of supposed primary tuberculosis 
of the central nervous system must be received with con- 
siderable doubt. I have had direct proof of tubercular in- 
fection, not only of the brain, but also of the cord, in vari- 
ous patients in the Montefiore Home, where, unfortunately, 
cases of chronic nervous disease are in immediate contact 
with those suffering from phthisis. A boy who had an old* 
hemorrhagic cyst developed a huge solitary tubercle from 
the walls of this cyst (Fig. 150). 

In size the solitary tubercle may vary from that of a pea or cherry to one 
as large as that depicted in the annexed figure. In this brain it will be seen 
that a solitary tubercle had occupied the greater part of one hemisphere, and 
had considerably distorted the entire brain axis. Very often the tumor starts 
in the vicinity of the meninges, or at least near the cortical blood-vessels. 
The blood-vessels unquestionably play an important part, for in cases of tuber- 



570 



THE NERVOUS DISEASES OF CHILDREN. 



cular meningitis of the base smaller tubercles of a pin-head size may be seen 
scattered along the vessels on the outer surface of the brain. A solitary 
tubercle is composed of a thick stroma with giant cells and a considerable 
amount of hard fibrous tissue . There is a more or less concentric arrange- 
ment of the parts, and the central portion of the tumor, in many cases, un- 
dergoes caseous degeneration at an early day. As in the cord, so in the brain, 
the tissue surrounding a tubercular growth may break down and become an 
almost diffluent mass. A tubercular encephalitis, or meningo-encephalitis, 
may, therefore, be superadded upon a solitary tubercle. In just as many 
cases, however, the solitary tubercle behaves as every other cerebral tumor 
does. It is, as a rule, sharply differentiated from the surrounding tissue, and 




Fig. 150. — Vertical Section through Cyst, C, and Both Tumors, showing Distortion of 
Brain Axis and Displacement of Left Ventricle. P, pons j V % the left ventricle. 



the exact character of it can be made out only on histological examination. 
The very size of the tumor, as in the brain depicted in Fig. 1 50, is suspi- 
cious of solitary tubercle, for the smallest as well as the largest tumors occur- 
ring in the brain are of this character. In the histological examination of the 
tumor search should be made for the tubercle bacilli, and their presence will 
at once prove or disprove the supposed character of the tumor. 

Glioma and sarcoma, or a combination of both, are the 
next most frequent forms of neoplasm occurring in the brains 
of children. The glioma possesses the characteristics of neu- 
roglia tissue. Its growth is extremely slow, and for this 
reason it may be suspected in cases in which all the symp- 
toms point to a period of growth lasting for one or two 



TUMORS OF THE BRAIN AND ITS MENINGES. 57 1 

years, or even a longer period of time. In connection with 
glioma, as in other forms of tumor, there is, as a rule, a con- 
siderable increase in the blood-supply to surrounding parts. 
An increase of cerebro-spinal fluid is a constant accompani- 
ment, and it must be due to variations in the amount of 
these fluids that improvement sometimes follows upon treat- 
ment by iodides and mercurials. 

A patient of mine had been suffering for a long time from symptoms point- 
ing to a tumor in the parietal region ; he had also had an apoplectic attack some 
years previously. Taking these facts into consideration I concluded that the 
tumor was probably of a specific character, and placed him upon a very rigid 
iodide and mercurial treatment. The improvement was most marked, the 
headaches ceased, the convulsive seizures disappeared altogether, and 
the power in the right hand returned. I was convinced of the specific 
character of the tumor. The patient died, however, in a relapse, accompanied 
by an apoplectic seizure, and to my great surprise, on post-mortem examina- 
tion, the tumor, which was examined by Dr. Van Gieson, proved to be a 
glioma and not a gumma. It is of the utmost importance, therefore, not to 
attach too great significance to any improvement occurring in these diseases 
after specific treatment. 

Gliomata occur more frequently in the white than in the gray matter of the 
brain, but may invade the latter though starting from the white substance. 
The pons shares with the hemispheres the distinction of being the most fre- 
quent seat of glioma, the dense character of the tissue in the pons evidently 
yielding favorable conditions for the development of this special form of 
neoplasm. 

Sarcoma, pure and simple, or in connection with glioma, is somewhat less 
frequent than glioma alone. Its growth is more rapid than that of simple 
glioma, and it is not as distinctly separated from the healthy tissue as glioma 
generally is ; the tumor and infiltration as a rule extend for some distance into 
the neighboring tissue. By this invasion and by actual compression much 
of the tissue in the vicinity of a sarcoma is readily broken down. Round 
cells and spindle-shaped cells occur together with the fibrous mass, and 
according to the predominating character of the cells the sarcomata are di- 
vided into round-celled and spindle-celled varieties. Myxo-sarcoma is not 
unknown in childhood, and a few cases have been reported of that much 
rarer form known as melano-sarcoma. The sarcomata are invariably secon- 
dary to similar diseases in other organs. Sarcoma may therefore be sus- 
pected with malignant disease in other parts of the body, and particularly if 
the tumor be one of rapid growth, and if the symptoms point to an intensely 
destructive process. 

Cysts are extremely frequent in children, and are often found in brains 
not suspected of any gross disease. Such cysts are generally the remnants 
of an acute process early in life, an early hemorhage, or a softening due to 



572 THE NERVOUS DISEASES OE CHILDREN. 

embolism or thrombosis. , These cysts fill up with fluid, and cause in the 
majority of cases no symptoms such as we assign to tumor ; but if from any 
cause such an old cyst is lighted up and its contents suddenly increase, the 
symptoms may resemble those of cerebral neoplasm. It is well to be pre- 
pared for the occurrence of such cysts, and to know that they are a favorite 
starting-point for other tumors. It was on the strength of such familiarity 
with the cerebral conditions of childhood that I was able to make the diag- 
nosis of a tumor growing from the cyst-walls in the case referred to on page 
569. 

Cystic tumors, in the true sense of the word, are those which are due to 
an invasion of echinococcus. In this country it is extremely rare to find a 
tumor of this description, but in Europe and in Eastern countries they 
are far more frequent. The cysticercus cysts occur in children as well as in 
adults, are multiple, and may give rise to a confusion of symptoms. 

The only other form of neoplasm which deserves special 
consideration is that due to syphilitic disease Gummata 
are unquestionably rare in children, but they may of course 
occur in cases of hereditary syphilis. We should look for 
them in connection with specific disease of the brain and 
its coverings. Repeated attacks and relapses would sug- 
gest the syphilitic character of the disease. 

Aneurism is also so rare in childhood that it would hardly need to be 
considered. But Osier reported some years ago an interesting case of aneu- 
rism in a child six years of age. The symptoms are very much like those 
caused by ordinary neoplasm, but the course of the disease is much 
more chronic than in the case of other new-growths. The diagnosis of 
aneurism could be reached only by exclusion, by the slow course of the dis- 
ease, and possibly by irregularities of the pulsations of the two carotid arte- 
ries. The anterior and posterior cerebral arteries are the vessels favored by 
aneurisms of any considerable size. 

In concluding these remarks upon the pathology of 
tumors, it is well to remind the reader once more that the 
symptoms produced by any tumor are in part due to the 
tumor itself, to the character of the invasion, and to the 
region of the brain affected, and in part to the pressure 
upon the neighboring tissues, and upon distant parts of the 
brain structure. The increase of intra-cranial fluid plays a 
most important part in the causation of some of the symp- 
toms, notably of headaches and of optic neuritis. 

Treatment. — Little is to be expected in children from 
medicinal or surgical treatment in cases of cerebral tumor. 



TUMORS OF THE BRAIN AND ITS MENINGES. S73 

The results, even in the adult, have not been in the least en- 
couraging. Thus Bramwell states that out of twenty -two 
cases of tumor of the brain in the adult successful removal 
would have been impossible in seventeen. In children the 
prognosis is still gloomier. For this there is a double rea- 
son ; first, tubercle is very much more frequent in children 
than in adults; second, if the conditions for the removal 
of the tumor are favorable, the child stands a poorer chance 
of surviving the operation than the adult does. Before pro- 
ceeding to the surgical treatment of the case of brain tumor, 
which is, after all, the only thoroughly rational course, the 
attempt should be made to influence the growth, and to 
promote the absorption of the new-growths by various 
drugs. The most powerful drugs are the iodides and the 
mercurials. Gummata are very rare in children, and this 
treatment would therefore be of little use if it were given 
for its anti-syphilitic effect. But in every case of tumor 
there is considerable exudation, considerable increase of 
cerebro-spinal fluid, and both the iodides and the mercurials 
seem to have some effect upon the absorption of these prod- 
ucts of inflammatory reaction in connection with tumor. 
By promoting such absorption of adventitious products, 
the intense localized headaches and persistent vomiting, 
and other general cerebral symptoms frequently present, 
are modified. Another reason for the employment of spe- 
cific remedies is the belief that if they will not avail any in 
the actual cure of the trouble, they Avill at least help to re- 
lieve some of the most distressing symptoms that occur in 
connection with cerebral neoplasm.* 

If any decided effect is to be hoped for, the iodides, as well as the mercu- 
rials, should be given in effective doses ; the iodides in doses varying from 
five to twenty-five grains three times a day ; the mercury, in the form of 
inunctions, from fifteen to thirty and forty-five grains twice a day. ac- 
cording to the age of the child. All other medicinal treatment will be of no 
avail. 

The headaches will be relieved best by a combination of phenacetine with 
codeine (one-fourth to one-third of a grain), or with small doses of morphia, 
from one-sixteenth to one-eighth of a grain, according to the age of the child. 
Morphia alone I am not in favor of giving ; and if the pains are so ex- 

* Quincke's method of lessening intra-cranial pressure by a lumbar puncture might 
be tried in inoperable cases. Trephining for the relief of pressure is justifiable. 



574 THE NERVOUS DISEASES OF CHILDREN 

treme that the child becomes restless and sleepless in consequence of them, I 
should prefer moderate doses of chloral hydrate, from five to ten grains, ac- 
cording to the age of the child, given either per mouth or per rectum. 
If the tumor causes frequent convulsions the bromides might be added 
to the chloral, but it is best to avoid them in all cases in which epileptic seiz- 
ures do not occur, for the bromides have no effect upon the intense headaches, 
unless given in very large doses, and have a disagreeable effect, interfering 
with the digestion and nutrition of the child. The ordinary hypnotics, such 
as sulfonal, trional, chloralamid, and the like, can be employed in moderate 
doses in those cases in which there is a general restlessness without pain. 

A few years ago great hopes were entertained that tu- 
mors of the brain would be successfully removed by surgi- 
cal means. These hopes have been but poorly realized in 
the cases of cerebral tumors of the adult, and with regard to 
tumors in the child the reported cases are so meagre that 
very few positive conclusions can yet be drawn. The diffi- 
culties of surgical interference in children are, in part, simi- 
lar to those met with in the adult, and in part peculiar to 
children only. Among the first we may mention the uncer- 
tainty which still prevails in every case regarding the exact 
size of a tumor. Although the symptoms may point to the 
strict localization of the tumor we cannot, except in the few- 
est cases, form any definite opinion before operation either 
as to the size to which the tumor has grown, or as to the 
exact extent of it on the surface, particularly if the tumor 
began in one of the active areas of the brain, and spread to 
a so-called silent area. On this account alone, however, no 
one would be justified in refraining from an operation; this 
uncertainty constitutes one of the attending risks. 

Among the conditions peculiar to children I would mention first of all the 
fact that children, according to my experience, which has been quite exten- 
sive, tolerate operations upon the brain very poorly indeed. The cerebral 
shock, and, above all the considerable cerebral hemorrhage, lessen the chances 
of recovery very much. If we consider, moreover, that a very large number 
of the cerebral tumors in children are of a tubercular nature, that multiple 
tumors are not infrequent, that the tumors occur with great frequency in 
the cerebellum and on the ventral surface of the brain, we are forced to the 
conclusion that there are but few cases of intra-cranial tumor in children 
which seem favorable for operation, and that of these few it must be a rare 
fortune indeed to have a single one survive the operation, or to recover from 
the invasion of the tumor. I do not, however, wish to give too gloomy a 
prognosis as regards the effect of operation, and believe it to be the duty of 



TUMORS OF THE BRAIN AND ITS MENINGES. $7 5 

every careful physician to weigh the evidence for and against an operation in 
every case, and if in a child of good physical strength a non-tubercular tu- 
mor is suspected in a region accessible to the surgeon's knife, the attempt 
at removal of such a tumor is justifiable if all other remedial measures have 
been given a previous trial. Whether a tubercular tumor should be removed or 
not is a question that cannot be decided off-hand. The answer may possibly 
be given some day by the results of operation in a case in which the charac- 
ter of the tumor was not suspected until it had been successfully or unsuc- 
cessfully removed.* While I am thoroughly opposed to cerebral surgery of 
the experimental order, repeated operations may be warranted, as in one case 
reported by Erb. The unusual success of such men as Kocher, Horsley, and 
Czerny leads one to hope that something may yet be done for the relief, 
and possibly for the cure, of this sad class of cases. 



BIBLIOGRAPHY. 

Bernhardt: Monograph. Berlin, 1881. 

Bramwell : Intra-cranial Tumors, 1888. Remarks on Intra-cranial Surgery 

(Reprint). Edinburgh, 1894. 
Christ : Archiv. f. kl. Med., vol. xlvi., p. 497. 

Dercum : Cerebellar Tumors, New York Medical Journal, August, 1892. 
Erb : Zur Chirurgie der Hirntumoren, Deutsche Zeitsch. f. Nervenheilk., II., 

p. 414, 1892. 
Knapp : Intra-cranial Growths. Boston, 1891. 
Kocher: Deutsche Zeitschr. f. Chirurg., vol. xxxvi., h. 1 and 2, 1893. 
Macewen : British Medical Journal, 1888. Infective Diseases, etc., 1893. p. 

146. 
McBurney and Starr : American Journal of the Medical Sciences, 1892. 
Mills : Article in Pepper's System, vol. v. 
Nothnagel: Top. Diagnostik, etc., 1879. 
Park : New York Medical Journal, November, 1889. 
Peterson, F. : In American Text-book of Diseases of Children. Philadelphia, 

1894. 
Sachs : American Journal of the Medical Sciences, March, 1891. New York 

Medical Journal, 1891. 
Seguin : Cerebellar Tumors ; Journal of Nervous and Mental Disease, 1887. 
Starr: Medical News, vol. lxiv., 29. Brain Surgery, 1893. Article in Keat- 

ing's Cyclopaedia. 
Steffen : Gerhardt's Handbuch, etc., vol. v., 1881. 
Stieglitz and Gerster : American Journal of the Medical Sciences. June, 1892. 

* Since this was written v. Beck (Beitrage zur kl. Chirurgie, vol. xii., 41) has re- 
ported a case of tubercle of the brain successfully removed by Czerny. The author re- 
ports improvement after the operation. Dana has very recently exhibited a K\\ of 
about twelve years who had done well for more than a year after the partial removal 
of a sarcoma. 



CHAPTER XXX. 

ABSCESS OF THE BRAIN. 

An abscess may be situated either on the surface of the 
hemispheres, or within the substance of the brain. The 
latter location is by far the more frequent, as the gray 
matter seems less liable to undergo suppuration than the 
white matter. Multiple abscesses sometimes occur. In one 
case the author observed at least six abscesses, which were 
successively incised; but a single abscess is more common. 
If the pus is on the surface of the brain the membranes, 
which are generally thickened as the result of the inflam- 
matory process, may constitute one wall of the abscess. 

Abscess of the brain may occur at any period of life. Of the cases which 
Gowers has tabulated fully one-third occurred before the age of nineteen. 
The male sex is more disposed to this disease than females, in a ratio vary- 
ing from two to one to five to one, according to the initial cause. The most 
frequent cause of abscess of the brain is preceding ear disease. Next in 
frequency is traumatic injury to the skull. Gull and Sutton, in their article 
on abscess of the brain, in Reynolds's " System of Medicine," state that 102 
cases of abscess were due to ear disease, and 57 to injury. Koerner, in a 
recent admirable monograph on the otitic diseases of the brain, quotes Pitt's 
statistics, who records 56 cases of brain abscess among 9,000 autopsies. Of 
these 56 abscesses 18 (very nearly one-third) were due to disease of the 
ear and of the petrous bone, while 8 (one-seventh of the entire number) 
were due to disease of other cranial bones. The relative number of other 
cerebral conditions complicating ear disease, as given by Koerner, is as fol- 
lows : Phlebitis and thrombosis of the sinuses occurred in 44 cases among 
9,000 autopsies, and of these 44 fully one-half were due to disease of the bony 
parts of the ear. A simple otitic meningitis as the result of purulent ear 
disease is relatively rare. Taking all cases of cerebral disease complicating 
otitis into consideration Koerner states that of 246 cases which he has been 
able to collect, 44 occurred before the age of ten, and 73 between the ages of 
eleven and twenty years. Thus it will be seen that fully one-third of all 
abscesses occurred during the period of childhood and early youth. Koerner 



ABSCESS OF THE BRAIN. $77 

states, basing his assertions upon Prussian statistics, that 5.15 per cent, of 
all deaths between the ages of ten and twenty were due to cerebral disease 
complicating otitis. These statistics will suffice to indicate the great inter- 
est that must attach to suppurative disease in early life, and particularly to 
abscess of the brain following upon disease of the ear. Other probable 
causes of abscess must not be neglected. Disease of the nose plays an im- 
portant part, and traumatic injury to the skull, whether or not it has given 
rise to a tangible bone lesion, may be the starting-point of brain abscess. 

Gowers states that fifteen per cent, of all the cases of abscess of the brain 
were due to what he calls distant influences. Among these any condition 
giving rise to a general infection may be included, and it is more than prob- 
able that even among this fifteen per cent, of cases some may have been 
traumatic, although the exact character of the traumatism could not be de- 
termined. 

In abscess of the brain following upon chronic otitic 
disease the ear trouble may have existed for several years. I 
have known such ear disease to continue for ten years before 
the symptoms of abscess were developed. As long as there 
is a free discharge of pus outward, the danger of cerebral 
disease is comparatively slight ; but if the discharge is 
checked, the pus may burrow its way through the thin 
plate of bone separating the middle ear and the mastoid 
cells from the cranial cavity. The small veins which pass 
through the ear structures into the cerebral sinuses (supe- 
rior petrosal and the lateral sinus) provide easy means of 
access for the pus from the inner ear into the cranial cavity. 
From the mastoid cells the pus passes most easily into the 
lateral sinus. In connection with ear disease the abscess 
generally forms either in the temporo-sphenoidal lobes or 
in the hemispheres of the cerebellum. Usually it affects 
these regions, on the same side of the brain. In rare in- 
stances the chief formation of pus is on the side opposite to 
the diseased ear. 

The abscess formed in connection with ear disease is 
more often underneath the gray matter of the cortex than 
upon the outer surface of the hemispheres, and not infre- 
quently normal tissue separates the abscess from the carious 
bone and the meninges. Such being the case, it is evident 
that the abscess must have been formed within the brain 
and gradually extended outward. In a few instances a di- 
rect path of connection between the suppuration in the ear 
37 



57^ THE NERVOUS DISEASES OF CHILDREN. 

and the abscess cavity has been demonstrated, but in the 
vast majority of cases no such connection has existed. The 
problem, therefore, is to decide in what manner the pus- 
corpuscles could have been carried from the ear to the in- 
terior of the brain. No entirely satisfactory solution has 
as yet been given, but the frequent interference with the 
circulation in the sinuses undoubtedly plays an important 
part. If we take into account the relative position of the 
temporo-sphenoidal lobes and the structures of the ear it 
will be seen that the superior petrosal sinus receives blood 
from both these parts, and, on the other hand, the lateral 
sinus receives blood from the cerebellum and the mastoid 
cells. It is due to these venous connections that abscess of 
the cerebellum is met with more frequently in connection 
with mastoid disease, and abscess in the temporo-sphenoidal 
lobes more frequently with disease of the middle ear. For 
the present we can do no better than to suppose that the 
septic material is carried along these sinuses. 

Abscess of the brain occurring in connection with diseases of the nose 
must be explained in much the same way. The nasal disease is sometimes 
limited to the mucous membrane without any involvement of the bone, yet 
abscesses form just the same. Under such conditions the toxic substance 
must be carried along the blood-vessels or the lymphatics. If the nasal 
bones are the seat of suppuration, as in syphilis, the abscess that forms may 
be in direct connection with the nasal cavity, and the pus from the brain may 
be freely discharged through the nose. 

The breaking down of tubercular growths may occasionally be the cause 
of abscess, but these cases have less practical interest, for the general tuber- 
cular character of the disease is the significant feature, and the abscess, as a 
rule, a mere incident in the course of a protracted illness. 

Purulent disease in any other part of the body may also be the cause of 
abscess of the brain through the agency of a septic embolus. Gowers quotes 
Boettcher's report of a case in which a cerebral abscess secondary to suppura- 
tion in the lung was found to contain lung pigment. There is a remarkable 
connection between brain abscess and every form of purulent disease within 
the thoracic cavity. Thus we have cases of brain abscess after pneumonia 
in which the exudate has not been perfectly resolved and has undergone sup- 
puration. Pus cavities occur after purulent bronchitis and empyema, but 
they rarely if ever result from tubercular cavities, while the occurrence of 
tubercular meningitis in connection with such cavities is quite frequent. 

As a rare cause of abcess, but one that is worth mentioning, we may note 
its occurrence after thrush in two cases reported by Zenker. One patient was 
an infant. In these cases the brain was studded with small abscesses con- 






ABSCESS OF THE BRAIN. 579 

taining the fungus of thrush (oi'dium albicans) ; the fungus had evidently 
travelled by way of the blood-vessels, since it had actually been demonstrated 
in their interior. 

Symptomatology. — Few conditions are more difficult to 
recognize during life than abscesses of the brain. It is safe 
to say that they constitute a large proportion of the sur- 
prises and disappointments of the post-mortem table. They 
are found when least expected, and when confidently looked 
for are often wanting. The uncertainty in the diagnosis is 
due to the very insidious development of the process and 
to the fact that when once encapsulated a pus cavity gives 
rise to very few symptoms. 

When fully developed an abscess practically constitutes 
a foreign body in the brain ; we would therefore expect 
such symptoms as are commonly due to tumors within the 
brain or cranial cavity, but in this we are apt to be disap- 
pointed, for the brain appears to accommodate itself to the 
slowly increasing abscess and does not respond to this 
growth as distinctly as it does to the invasion of a solid 
neoplasm. The abscesses do not, moreover, occur as fre- 
quently as tumors do in those parts of the brain which give 
rise to signal symptoms (the motor area and the occipital 
lobes). Abscesses are more common in the frontal lobes, in 
the temporo-sphenoidal lobes, and in the cerebellum. In 
acute cases, favorable for diagnosis, the first symptoms are 
those with which we are familiar in connection with menin- 
geal disturbances, such as local painfulness of the scalp, 
nausea, vomiting, vertigo, and fever. The last, if irregu- 
lar and if attended by rigors, is the one symptom which 
suggests abscess much more forcibly than any other. If 
the cavity is in the vicinity of the motor areas and is not 
too rapidly destructive, localized convulsions may occur. 
Abscess in this region, which is supplied by the middle 
cerebral artery, is very likely to be due to septic embolism ; 
hence the formation of pus in this region after lung disease 
and after septic endocarditis. Paralysis may be present in 
some of these cases if the motor area is involved, but if the 
abscess is elsewhere in the brain paralytic symptoms will 
not constitute a prominent feature of the disease until the 
terminal stage has been reached. 



580 THE NERVOUS DISEASES OF CHILDREN. 

To this category of symptoms, delirium, convulsions, and 
unusually high temperatures may be added, which lead to a 
rapidly fatal issue. 

Chronic abscess may exist for a number of years and 
may give rise to very few symptoms ; in one case of chronic 
abscess, in a boy of eight years, no symptoms were pres- 
ent as long as the ear freely discharged. As soon as this 
ceased, epileptiform convulsions, preceded by sensory 
auras (disagreeable odor and a hissing noise) would occur. 
This alternation was kept up for at least two years, when 
the boy died of a general purulent meningitis from bursting 
of the abscess into the temporo-sphenoidal lobe. The so- 
called latent state of the abscess is characterized, as a rule, 
by occasional spells of nausea, by occasional vomiting, and 
by intermittent febrile attacks for which no proper expla- 
nation can be found at the time. Persistent headaches, if 
associated with ear disease or with other conditions that 
give rise to abscess, point to the existence of this condition. 
The headache is generally more or less circumscribed, and 
sometimes varies according to the position of the head ; but 
this mere fact of change of position as affecting the head- 
ache does not necessarily point, as one would suppose, to 
abscess, for I have known the same condition to accompany 
solid tumor of the brain. The pain, as a rule, is referred 
to the vicinity of the abscess, but not invariably so. Mac- 
ewen's symptom (as described in Chapter XXIX.) may be 
of service, but should be utilized with great caution. The 
temperature is often lowered in abscess of the brain, and is 
accompanied by slow pulse. (Macewen.) 

Optic neuritis, so characteristic of tumor, also occurs in 
abscess of the brain. It is not as reliable a symptom in the 
latter as in the former condition ; yet it is much more fre- 
quently present than was formerly supposed to be the case. 
The neuritis is in nowise to be distinguished from the 
neuritis associated with ordinary tumors within the cranial 
cavity. The other symptoms will vary very much accord- 
ing to the location of the abscess. Paralysis, if present, is 
apt to be hemiplegic. If associated with unilateral con- 
vulsions it points to the motor cortex as the site of the 
abscess. Sensation is, as a rule, not involved, and the 



ABSCESS OF THE BRAIN. 58 1 

various cranial nerves are not affected unless the abscess 
is at or near the base. The fifth and sixth nerves may 
be pressed upon by a cerebellar abscess, causing- pain in 
the face and paralysis of the muscles of mastication and 
of the rectus externus. Cerebral abscess may also be asso- 
ciated with paralysis of the facial nerve, but this is only 
rarely due to involvement of the nerve as it emerges from 
the pons, but is more frequently of old standing and due to 
an early disease of the ear affecting the nerve in its course 
through the Fallopian canal. The various forms of aphasia 
may be present if the abscess involves parts connected with 
the function of speech. In a patient whom I had the privi- 
lege of seeing with Dr. Gruening, sensory aphasia was the 
first signal symptom of an abscess in the temporo-sphenoi- 
dal lobe. Hemianopsia may be present (case of Knapp). 
Stupor, delirum, and coma mark the terminal stages of 
abscess. 

The first suggestion of abscess comes occasionally 
through the sudden and vehement change in the symp- 
toms, this change being due to the rupture of the abscess 
into the ventricles. 

Differential Diagnosis. — From the account of the 
symptoms accompanying abscess of the brain it is evident 
that it is difficult, sometimes impossible, to differentiate 
between a pus cavity in the brain and other conditions. 
Among these are solid tumor of the brain, meningitis, and 
meningo-encephalitis. The diagnosis of solid tumor can 
more readily be made if the usual causes for abscess, such 
as ear disease and traumatic injury are wanting, and if the 
symptoms have developed in a subacute fashion, which 
is rather characteristic of tumor, while in abscess there is 
either a prolonged latent period or all the symptoms de- 
velop in a very short period of time. Suspicion of abscess 
is strengthened above all things by the presence of marked 
rigors and rapidly changing temperatures. A slight change 
of temperature may occur in the case of tumors, but it is 
not as variable and does not show the extremes so common 
in abscess. During the past year I have observed throe 
cases of abscess in which there was not the slightest rise 
of temperature for several weeks preceding death. 



582 THE NERVOUS DISEASES OF CHILDREN'. 

The differential diagnosis between tumor and abscess is one of practical 
importance. If the new-growth is near the surface, and if the symptoms are 
of such a kind as to lead one to infer a tumor of considerable extent, the 
surgeon might well hesitate to operate if he thought the new-growth a solid 
one. If there is a probability of an abscess the size of the abscess need 
not constitute a contraindication to operation, and this differentiation be- 
tween tumor and abscess is still more important if the growth is supposed to 
be in the interior of the brain. If this should be a solid tumor a surgical 
operation would do no good, but if it is an abscess it could well be drained 
through the hemispheres, and the greatest danger of all, rupture into the ven- 
tricles, might be averted. 

The differentiation between meningitis and abscess is not an easy one, par- 
ticularly in cases of ear disease and external injuries. Meningitis is also apt to 
be associated with or to precede the formation of abscess, and during this 
period the question whether the suppurative, and hence the operative, stage 
has been reached is not always easy to decide. Moreover, in the final stages of 
abscess, particularly if the abscess has ruptured, a general purulent meningitis 
is the invariable result. At that stage a differential diagnosis can easily 
be made with a great degree of certainty, but if made has little practical 
value. In the earlier stages of cerebral disease after otitis or external injuries 
it is safe to believe that the condition is still one of meningitis, without forma- 
tion of abscess, if the symptoms are those of a general cerebral disturbance, 
such as vertigo, headaches, nausea, slight stupor, but without rigors or serious 
rise of temperature. Localizing symptoms may be present in both cases. 

The distinction between meningitis and abscess is of much practical im- 
portance for another reason. The majority of surgeons would not be willing 
to operate upon cases of meningitis ; they evidently have the general puru- 
lent meningitis in mind ; with improved methods in surgery, exploratory 
trephining may be warranted, particularly in connection with ear disease or 
external injury if there is a suspicion of meningitis alone, provided the latter 
be limited in extent. 

In practice the differential diagnosis most frequently to be established is 
between abscess and sinus thrombosis after middle-ear disease. The gen- 
eral cerebral symptoms may be similar in both conditions, but the special 
symptoms of sinus thrombosis (see p. 586) will help to decide the point. In 
many cases the condition is uncertain before the operation is undertaken. In 
every doubtful case the surgeon should look first for sinus thrombosis, and 
then for abscess. 

Prognosis. — The prognosis of abscess of the brain is in- 
variably grave. While admitting the possibility of an ab- 
scess becoming encapsulated and of remaining latent in the 
brain for many years, the probability is in every instance 
that serious mischief will result from it sooner or later. 
The physician must not trust to good fortune in such dis- 
eases, but knowing the serious nature of the trouble he is 



ABSCESS OF THE BRAIN. 583 

bound to present to the patient, or the patient's relatives, 
the alternatives of surgical interference or death within a 
limited period of time. The longer the abscess has lasted 
the more serious it is apt to be, for the danger of rupture 
becomes greater with the increase in its size. 

Treatment. — In spite of recent advances in cranial sur- 
gery and the little that has been accomplished in the treat- 
ment of abscess of the brain it is best to discuss the proph- 
ylactic measures which may possibly prevent the formation 
of abscess. Knowing that pus is formed most frequently 
after ear disease and after disease of the bone, the greatest 
caution should be exercised to prevent any extension of the 
inflammatory process into the cranial cavity from these 
original sources of infection. The possibility of cerebral 
complications in every case of suppurative ear disease 
should be kept well in mind, and free discharge outward 
should be secured and maintained. In the case of mastoid 
and middle-ear disease we are not only bound to secure a 
free passage outward for the pus, but the discharge should 
be watched, and as soon as the pus ceases to flow outward 
while the symptoms point to a constant generation of pus 
in the bony structures, surgical measures should be resorted 
to to drain away the pus that is formed. While pus is dis- 
charging freely from the internal or the middle ear, if symp- 
toms arise pointing to meningeal or cerebral involvement, 
such as increasing headaches, vertigo, vomiting, and fever, 
further surgical aid is needed. 

An attempt may be made when the first meningeal 
symptoms arise to treat these in the ordinary way by the 
application of cold, by blistering, by counter- irritation, 
and the application of leeches and the like, but very little 
time should be wasted with such measures, and if the 
symptoms do not yield within twenty-four or forty-eight 
hours the surgeon must afford the needed relief. In the 
case of abscess due to ear disease, until recently little suc- 
cess has attended the efforts of the surgeon to locate and 
drain the abscess; but it bids fair to become the most satis- 
factory and legitimate cause for operation upon the brain. 
Such abscesses are most frequently situated in the first and 
second temporal convolutions. 



584 



THE NERVOUS DISEASES OF CHILDREX. 



These convolutions can be easily exposed by trephining 
at a point which is reached by drawing a line one and a 
quarter inch back from the external auditory meatus, and 
drawing another, at right angles from this one, the same 
distance upward. The terminating point of this line may 
be made the centre of the trephine opening, and will be 
sufficiently accurate to enable the surgeon, after removing 
a considerable portion of the bone, to expose the region 
of the abscess. As the abscess is not always superficial, 
punctures should be made with a fine hypodermic needle in 
various directions in order to reach cavities that are situated 

below the grav mat- 
ter. By observing 
these rules one can 
hardly fail to find the 
abscess. In one case 
of the author's, the 
surgeon had to make 
four punctures into 
the substance of the 
brain before pus was 
withdrawn. Absces- 
ses formed in connec- 
tion with disease of 
other parts of the cra- 
nium can generally be found if one is guided bv the external 
signs ; thus, in a case which was operated on for me about 
six months ago, there was caries in the middle of the parietal 
bone. The surgeon was directed to open the skull at this 
point, and, if no abscess presented, to insert the needle in 
various directions. When the dura was exposed nothing ab- 
normal was noticed, but the first thrust of the needle secured 
a large quantity of pus : the brain tissue was then incised 
and the entire abscess cavity opened, but the patient died 
a few days later, and on the post-mortem table other ab- 
scesses were found in the opposite hemisphere of the brain 
as well as at the base. The same rule should be followed 
in cases of abscess following upon traumatic injury of the 
skull. The trephine opening should always be made at the 
seat of the external injury, and enlarged from this point to 




€.a.?n 



Fig. 151. — Dissections showing the Guide Adopted 
by Barker in Successful Trephining for Abscess 
from Ear Disease. (After Gowers. ) 



ABSCESS OF THE BRAIN. 585 

meet the exigencies of a given case. If the pus cavity be 
due to disease of the nose, a large opening in the frontal 
bone should be made, from which the surgeon will then 
attempt to locate the exact seat of the abscess, and, if possi- 
ble, drain it according to the usual method. 

Abscesses in the cerebellum and at the base of the brain 
are generally beyond reach, though it is to be hoped that 
the former may become accessible by improved surgical 
methods. 

Successful treatment of brain abscess has been reported by Stimson, 
Bergmann, Shede, Paget, Park, Koerte, and Knapp. The case of Knapp is of 
special interest, as it occurred in a child nine years of age. The chief symp- 
toms were chronic otorrhcea (after scarlet fever), optic neuritis, homonymous 
hemianopsia, and Macewen's symptom (percussion note stronger on left 
side) ; there was no sensitiveness on percussion of the skull. At the opera- 
tion a pus cavity was found, and complete recovery ensued in the course of 
several weeks, after minor mishaps. 

There is no reason why children who present indubi- 
table signs of brain abscess should not be operated upon ac- 
cording to the same principles which hold good in adults. 
It is probable that with greater experience in these cases 
the operation need not last several hours, as in some of 
those hitherto reported, and hernia cerebri can surely be 
avoided. I agree with Starr in his remark, that the earlier 
the surgeon is called in the better the chances of the pa- 
tient. 



THROMBOSIS OF THE INTRA-CRANIAL SINUSES. 

The blood circulates in the venous sinuses within the skull under special 
disadvantages. The walls of the sinuses are rigid, the blood cannot be emp- 
tied during inspiration, and many of the cerebral veins join the longitudinal 
sinus at an obtuse or right angle, so that their blood is poured into the su- 
perior sinus in a direction opposite to the main current. Under such condi- 
tions it is natural that the current should be retarded and coagulation of 
the blood easily set in. 

Two forms of thrombosis occur ; the first is the primary or marasmic 
thrombosis which occurs in children after exhausting diseases (cholera infan- 
tum, etc.). This is generally confined to the longitudinal sinus, but may in- 
volve the lateral and cavernous sinuses. In such cases the clots are dense. 
resistant, organized, and do not adhere to the wall of the vein. The throm- 



586 



THE NERVOUS DISEASES OF CHILDREN. 



bosis may be limited to the cerebral veins, thus giving rise to limited or cor- 
tical symptoms. Gowers, it will be remembered, has attached special signifi- 
cance to this condition in the causation of infantile cerebral palsies. 

The second form is the secondary or infective thrombosis, which generally 
affects the lateral, the cavernous, or the transverse sinuses. It is secondary 
to some infective process in the neighboring tissues or at a distance. Middle- 
ear disease is the most frequent cause, but it may also be due to traumatic 
injuries of the skull (infected wounds), erysipelas of the head and face, to 
purulent disease of the eyes and of the nose. In one of the author's cases 
caries of the jaw was the starting-point of sinus thrombosis. In other 




Fig. 152. — Girl, Aged Twenty. Exophthalmus, with internal strabismus ; oedema of 
the right eyelids, side of the nose, the brow, and the face. Later in the disease the 
left side was also affected. Thrombosis of cavernous sinus. (Macewen. ) 



cases the infectious material is carried along the veins and through neighbor- 
ing tissues. 

The inflammation may extend directly to the walls of the sinus and thus 
cause clotting of the blood within, or the clot may form within a vein and 
extend from there into the sinus. The superior petrosal and the lateral 
sinuses receive their blood from the middle ear, hence the frequency of throm- 
bosis of these sinuses in middle-ear disease. Thrombosis of the sinus result- 
ing from actual compression is very rare indeed and of little significance, as 
the symptoms would be obscured by those of the primary affection. 

Symptoms. — The symptoms of sinus thrombosis are often complicated 
by those of the primary disease. We must distinguish between general and 
special symptoms. Among the former manifestations the most important are 
intense headaches, somnolence increasing to stupor and coma, convulsions, 
slight rigidity of the neck, optic neuritis, rigors, accelerated or diminished 



ABSCESS OF THE BRAIN. 587 

pulse-rate, thready pulse, and fluctuating temperatures ; but most of these 
symptoms would suggest meningitis or encephalitis quite as readily, if the 
conditions favoring sinus thrombosis were not known to be present ; more- 
over, actual meningitis may be caused by thrombosis of the sinus, particu- 
larly if such thrombosis is of an infective nature. 

The special symptoms refer particularly to disturbances in the venous 
circulation and to areas of tenderness. Thrombosis of the superior longi- 
tudinal sinus is characterized by oedema of the scalp and distention of the 
veins in the parietal, frontal, and occipital regions. In infants the anterior 
fontanelle becomes prominent, and epistaxis is frequent ; squint is a rare oc- 
currence. Cavernous sinus thrombosis is rare (the author has seen two cases 
within the past year) ; the thrombosis is generally due to extension from other 
sinuses, from the petrosal or lateral sinus, or it may result from disease of 
the ophthalmic veins (Knapp) and abscess in the orbital cavity. In more 
than half of the cases the thrombosis spreads to the cavernous sinus of the 
opposite side. The special symptoms are exophthalmus, oedema of the eye- 
lids and root of the nose due to venous obstruction ; also ptosis, paralysis of 
rectus externus, and supraorbital pain, all due to compression of various 
nerves to the orbit running close to the sinus. (See Fig. 133.) "Choked 
disk " from obstruction of the retinal and ophthalmic vessels is present in 
some cases of cavernous sinus thrombosis. Defect of vision and amaurosis 
may be superadded. Some of these cases live five or nine months, but all 
end fatally. The author has a case now under observation in which the 
condition has remained practically unchanged for nearly two months. 

Thromboses of the petrosal and lateral sinuses are the most frequent, and 
the most important ; unfortunately they present very few special symptoms. 
In lateral sinus thrombosis there may be distention of veins and oedema over 
the mastoid process, and tenderness on percussion in this region, but the 
symptoms differ very little from those due to ear disease and localized men- 
ingitis, and all three conditions may be present. Inflammation of the veins 
leading from the lateral sinus occurs in cases of infective thrombosis. When 
these veins are inflamed pain may be elicited along the internal jugular vein, 
and over the upper third of the posterior cervical triangle. A difference in 
the sensitiveness of the two sides will be strong corroborative evidence of 
suspected thrombosis. Cervical abscess and enlargement of cervical glands 
may result from infective external thrombosis of the lateral sinus. 

The prognosis of sinus thrombosis is always grave. A fatal issue is to 
be expected, although death may be delayed for a period of several months. 
The only hope lies in successful surgical interference and in early preventive 
measures. (See remarks on abscess.) The lateral and transverse sinuses 
have been opened successfully and the clots removed.* Ballance. Sulzer, 
Schwartze, Parkin, Pritchard, and Cheadle have reported successful cases of 
this character. Parkin's and Cheadle's patients were respectively nine and 
thirteen years of age. Koerner has collected 20 cases of operation for sinus 

* The details of the operation should be studied in Macewen's treatise, page 307 
et seq. 



588 THE NERVOUS DISEASES OF CHILDREN. 

phlebitis following otitis; of these, 13 were cured and 7 died. Tying the 
jugular vein before removing the clot seems to exert a favorable influence upon 
the percentage of cures. Koerner states that there was recovery in seventy- 
five per cent, of the cases if the jugular vein was tied, and only fifty per cent. 
recovered if the vein was not tied. 

A more extensive experience and a far larger collection of cases will be 
needed to decide many mooted points, but the successful operations recorded 
encourage one to hope that sinus thrombosis (excepting possibly as part of a 
general pyaemia) will become an eminently curable disease. Exploratory oper- 
ations in doubtful cases are not only justifiable but imperatively demanded. 



BIBLIOGRAPHY. 

Ballance : Lancet, 1890, vol. i. 

Barker: British Medical Journal, December 11, 1886. 

v. Bergmann : Monograph on Surgical Treatment of Brain Disease, Berlin, 

1888. 
Boettcher : Virchow's Jahresbericht, 1869, vol. ii., p. 51. 
Dean : Lancet, 1892. 

Gluck-Baginski : Beri. kl. Wochenschr., No. 48, 1891. 
Gowers : Text-book, vol. ii., p. 470, second American Edition. 
Gull and Sutton : Reynolds's System of Medicine. 
Knapp : Archives of Otology, vol. xxiii., No. 3, 1894. 
Koerner: Die Otitischen Erkrankungen des Hirns, etc. Frankfort, 1894. 

(Excellent summary and original cases.) 
Parkin : Lancet, No. 11, 1893, p. 522. 
Pitt : British Medical Journal, 1890, vol. i. 
Pritchard, Cheadle : Lancet, March 4, 1893. 

Randall : Transactions of American Otological Society, vol. v., Part I. 
Ribbert : Berl. kl. Wochenschr., 1879, p. 617. 

Schede : Report by Truckenbrod, Zeitschr. f. Ohrenhk., 1886, vol. xv. 
Scheier, Koerte : Berl. kl. Wochenschr., September 4, 1893. 
Schwartze : Handbuch der Ohrenheilkunde, vol. ii., p. 844. 
Starr : Brain Surgery, 1893. 

Stimson : New York Medical Journal, March 30, 3891. 
Zenker: Bericht der Gesellsch. f. Nat. u. Heilkunde, 1861, p. 62. (Quoted 

after Gowers.) 



CHAPTER XXXI. 

DISEASES AND CONDITIONS DUE TO DEFECTIVE DEVELOP- 
MENT OF THE BRAIN. 

In this chapter the author proposes to collect the vari- 
ous types of disease due to defective or arrested develop- 
ment of the brain. Strictly speaking, the term disease 
should not be applied to conditions which are not due to an 
actual morbid agent, but are the result of insufficient devel- 
opment of the brain or of different parts of the central ner- 
vous system ; but if the term disease is used in the larger 
sense implied in departure from normal conditions we shall 
not be straining the point too much, for the conditions dis- 
cussed in this section present symptoms that are strikingly 
different from those met with in health. The subject-mat- 
ter of this chapter is necessarily arranged on an anatomical 
basis. 

LARGE DEFECTS. 

Gross structural abnormalities in the development of the brain are sub- 
jects for scientific curiosity rather than for clinical study. It will not be 
necessary, therefore, to devote very much space to this special class of cases, 
for although they help to adorn the shelves of anatomical museums they 
are, after all, of very little practical interest. The following are the chief 
forms under which gross defects in cerebral and cranial development have 
been noted : 

I. Cyclops or monopus, as the name tells, indicates a condition in which 
but one orbit exists in the middle of the face, and about in the position gen- 
erally occupied by the root of the nose. The size of this one orbital cavity 
varies according to the development of the eye. In some cases the eye is 
not present, and the orbital cavity is correspondingly small. The cavity at- 
tains its largest size if the two eyes are fused into one ; and in accordance 
with the single or double development of the eye, one or two optic nerves may 
be present. If no bulb has been developed there is also a corresponding de- 
fect of the optic nerve. The eyelids may be single or double, the nose, as a 



59° THE NERVOUS DISEASES OF CHILD REX. 

rule, is undeveloped, and its place indicated by something which simulates 
a short snout. The entire features are distorted, the mouth may be wanting, 
and the ears come closely together. 

The only reason that we have to consider this condition at all is that it is 
due to a defective development of the anterior cerebral vesicle ; the ganglia, 
too. are more or less deficient ; the corpus callosum may be wanting alto- 
gether. The parts of the brain coming from the middle and hind brain vesi- 
cle are, as a rule, better developed than the hemispheres are. 

Fortunately these cases are not viable ; they have therefore no clinical 
value whatever, and may well be relegated to the authors specially interested 
in monstrosities. 

II. The next most frequent and most complete anomaly of brain structure 
is the condition known as anencephalus or hemicephalus. The difference 
existing between the two conditions consists, as the names indicate, in the 
complete or partial absence of brain development. Steffen assumes, without 
decided evidence, that these departures from normal development are due to 
an inflammation of the meninges occurring during the foetal period, in conse- 
quence of which a large amount of cerebro-spinal fluid collects in the ventri- 
cles, in the meshes of the pia, and in the subdural spaces ; and that in conse- 
quence of this increased pressure upon the developing parts the normal 
growth of the brain is interfered with. There is no reason to dispute this 
view until some better opinion is offered. The only suggestion to be made is 
that the inflammation may have been preceded by disturbances of circulation, 
and if such disturbance of circulation is the important factor, we shall have 
the same cause operative in these cases of larger defects as in those smaller 
defects called porencephaly, which we shall study hereafter. In cases of an- 
encephaly or hemicephaly a very considerable portion of the skull may be ab- 
sent. There may be rudiments of the parietal, of the temporal, or of the 
occipital bones. In some cases a portion of the occipital bone is left, and 
this, together with the petrous portion of the temporal bone, provides a small 
groove in which some cerebellar rudiments may be lodged. The frontal bone 
is, as a rule, undeveloped. If present, it is very low. the eyes therefore pro- 
trude, the face points upward, and the jaws push forward. Instead of the 
roof of the skull, a thickened membrane may be all that is present. In other 
cases the bony defect may be covered by a tolerably normal scalp, which 
may be fused with the dura mater. 

If the skull is opened even* conceivable defect may be found. In the ma- 
jority of cases after removal of the bone, the thickened membranes present 
themselves, and if these are punctured a very large amount of fluid flows out, 
the membranes collapse, and further examination of the specimen may show 
nothing excepting the basilar structures, no trace of the hemispheres or 
any part of the frontal brain being present. 

PORENCEPHALY. 

The condition of porencephaly is much more interesting from even* point 
of view than the larger brain defects previously mentioned. The term por- 






DEFECTIVE DEVELOPMENT OF THE BRAIN. 5QI 

encephaly has been much abused. It has been made to embrace an entire 
absence of brain structure, or very minute defects in the brain, secondary to 
vascular lesions. It would be best to use the term in the way it was first 
used by Heschl, who invented it, and by Kundrat, who wrote the first exten- 
sive monograph on the subject. As the name indicates, it is practically a 
hole in the brain, not an entire absence of brain structure. For that reason 
there should be some evidence of development of that part of the brain in 
which the hole lies. This hole, or porus, may be the result of defective de- 
velopment, or it may be acquired in the latest period of fcetal life, or even in 
the first period of life after birth. It would be well to distinguish the truly 
congenital cases from the acquired cases, with the additional qualification 
that even the truly congenital cases may be acquired in the sense of being 
the result of some morbid process during the fcetal period. 



Congenital Porencephaly. — The defect may be in the 
anterior, in the middle, the posterior, or, in fact, in any 
part of the brain, but it is found much more frequently in 
the anterior and in the middle portion. Congenital poren- 
cephaly may be single or double. 



An extreme condition of porencephaly will be best illustrated by Figs. 153 
and 154, taken from Schultze's monograph, which he entitles " A Contribution 
to the Doctrine of Congenital Cerebral Defects." I select this case because it 
represents, as it seems to me, an extreme condition of porencephaly, and is 
only one stage short of a defective development involving a very large portion 
•of the entire brain. By reference to the figures it will be seen that the posterior 
half of the brain is tolerably well developed, but that the anterior half is 
practically nothing but a sac enclosed by a thick membrane. The defect in- 
cluded not only the two frontal and a considerable portion of both parietal 
lobes, but the greater part also of both temporal lobes. The posterior half of 
the brain, the cerebellum, and all the ganglia proved normal on microscopical 
examination. Schultze describes this brain very thoroughly, and it is of es- 
pecial interest inasmuch as its bearer lived to the age of five years. As far 
as any history may be depended upon, it was simply this, that the child 
appeared normal at birth, and was supposed to have moved its hands ; but 
this statement Schultze questions, and states that later on both arms 
were in a condition of flexion and adduction. The legs were flexed, and 
were in a state of spastic contracture and adduction. The child never had 
any convulsions, but was able to scream and to suckle. The father of the 
child noticed that there was no sign of intelligence, that the child would not 
take hold of anything, that it did not hear, and that it lay constantly with its 
eyes rolling about, and with its head retracted. The condition was. therefore. 
one of complete idiocy, with paralysis and contractures of both upper and 
lower extremities. The child grew weaker and weaker, and finally died of a 



592 



THE NERVOUS DISEASES OF CHILDREN. 



bronchitis. The parents of this child had six others, all of them healthy. 
The mother of the child was addicted to drinking. 

Schultze disputes the correctness of Kundrat's views, that porencephaly 
is the result of anaemic necrosis. This author insisted on the marked 
distinction between the congenital and acquired cases of porenceph- 
aly. If the porencephaly is of intra-uterine origin, the convolutions are so 
arranged as to radiate toward the margin, or toward the middle of the 
defect. If the defect is an irregular one, and the convolutions are not ar- 
rayed in any definite fashion the porencephaly is acquired after birth. In 



a. c. C 




P. c. c. 



Fig. 153. 



-Brain with Large Anterior Defect. A. C. C, anterior central convolutions ; 
P. C. C. , posterior central convolutions. (Schultze.) 



this question, as in so many others, there is some truth in views that appear 
to be distinctly divergent. I have no doubt that some cases of porencephaly 
are due to vascular lesions, and others to inflammatory processes which may 
possibly start from the meninges. The symmetrical defect in Schultze's case 
seems to me more likely to be due to an inflammatory meningeal process 
without exudation, at a relatively early stage of foetal development, than to a 
destruction secondary to vascular disease. In Schultze's case, moreover, the 
vascular apparatus was found to be entirely normal. The question arises, 
What the causes of an intra-uterine inflammatory process could possibly be ? 
If the mother had passed through any pyasmic condition, or had had some 
form of purulent disease during pregnancy the cause of such inflammatory 
meningitis or encephalitis would not be far to seek ; but in Schultze's, as in 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 593 

other cases, no such cause could be made out, and it will not do to speculate 
upon improbabilities. The readiest explanation is to assume, as many of the 
older French writers assumed with regard to various brain defects, that 
traumatism plays a very important role, and that traumatic encephalitis dur- 
ing the intra-uterine period may be the actual cause of some of these poren- 
cephalic conditions. Schultze mentions that spasmodic contractions of the 
uterus without any extraneous disturbance may be a possible cause of such 
traumatic changes in the fcetal brain. In his own case Schultze is inclined to 
consider the possibility of a degeneration or obliteration of some of the 




Fig. 154. — Same Brain as in Preceding Figure, Viewed from the Base. (Schultze.) 

blood-vessels, or even of an encephalitis, as a direct result of alcoholic ten- 
dencies of the mother. In the most recently reported cases of porencephaly 
of the parietal region there seems to be atrophy of all motor fibres from the 
cortex to the medulla oblongata. (In eleven of fourteen cases. — Anton.) 

Acquired Porencephaly is a condition due to early meningeal 
hemorrhage. This in turn is the result of compression of the skull during 
labor. The symptoms associated with these brain conditions will be found 
described under the heading of Cerebral Birth Palsies. The meningeal hem- 
orrhage is in many cases most severe under the parietal eminences, and over 
the motor areas. For this reason double spastic cerebral palsy is a natural 
result of a defect which develops in the region occupied by the motor cen- 
tres. This region is also supplied by the middle cerebral artery, which is 
most liable to all vascular disease. The porencephalic defect due to men- 



594 



THE NERVOUS DISEASES OF CHILDREN. 



ingeal hemorrhage may be present in both hemispheres, or it may exist in 
only one-half of the brain. The meningeal hemorrhage, if sufficiently se- 
vere, leads to a compression of the cortical substance ; atrophy of the cortex 
follows, upon this the substance shrinks considerably, and the cerebro- 
spinal fluid is increased either as a result of inflammatory action, or of an 
effort at compensation. The pia and the cortex are apt to become aggluti- 
nated, and thus local cystic 
conditions with extreme loss 
of substance may readily oc- 
cur, the cyst occupying the 
/T 'lr ^s """^ place which should be rilled 

f ' * m>- J* 41 " V by normal brain substance. 

V- - f Js~~* w—"^^ ^ Through the kindness of 

jm W" W*~ *r S ' ^ r ' P eterson tne author is 

jjHpp **~ / 3 enabled to insert an illustra- 

f JR~~^ Uf*~ \*\ f tion (Fig. 155) of the brain of 

a child that died at the age 
of twenty months. The child 
had been subject to convul- 
sions and had presented com- 
plete spastic paralysis of all 
four extremities ; the head 
measurements were extreme- 
ly small (circ. 32.5 ctm.). Over 
each hemisphere a large por- 
tion of the convolutions was 
wanting. The vacuum was 
filled partly by subdural fluid, 
and partly by the bulging of 
each ventricle, for there was also internal hydrocephalus. Peterson thinks the 
condition due to a double meningeal hemorrhage, but the defect corresponds 
so nearly to the distribution of the middle cerebral arteries that obliteration 
of these blood-vessels early in foetal life may have been the prime cause. 




Fig. 155. — Large Double Porencephalic Defect. 
Child lived to age of twenty months. (From a 
brain kindly furnished by Dr. Peterson.) 



MICROCEPHALOUS. 



In addition to the anomalies of brain structure in which 
one or more parts may be defective, we have to consider 
that condition in which the brain as a whole shows a de- 
fective development, failing to attain to the normal size. 
There is, as a rule, a correspondingly small development of 
the skull, and to this small head the term " microcephalus " 
is given. While a microcephalus is bound to harbor an ab- 
normally small brain, we must not forget that a skull of 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 595 

normal size may also contain a small brain, the cranial 
cavity under such conditions being filled up by an excess 
of fluid. Microcephalus is evidently the result of a num- 
ber of different processes. It has been a habit to explain it 
altogether on Virchow's theory, that the smallness of the 
skull was due to a premature ossification of the cranial 
sutures, which interfered with the proper expansion of the 
skull, and that in consequence of this stunted growth of 
the skull the growth of the brain was arrested. This ex- 
planation will hold good for a considerable number of cases, 
but I am firmly convinced not for all. We must allow also 
for those cases of microcephalus due to defective cerebral de- 
velopment during the intra-uterine or during the early extra- 
uterine period. For reasons which we cannot always fathom, 
the brain does not grow and the skull remains small. The 
children are small-skulled and small-brained. In another 
series of cases the microcephalus is, I am convinced, the 
result of early vascular lesions. I have in mind several 
cases of children who were born normally, whose cerebral 
capacity was on a par with that of other children up to 
the age of six, seven, or nine months. Then convulsions 
set in, probably resulting in extensive meningeal hemor- 
rhage, or, let us say, in an extensive pachymeningitis hem- 
orrhagica. In consequence of this the cortex is com- 
pressed, it undergoes atrophy and sclerosis, and remaining 
small the skull does not expand in normal fashion. Such 
children become microcephalic after the first year of life, 
or later. 

This leads us to a question which has been much discussed and unsatis- 
factorily answered, whether the growth of the skull depends upon the growth 
of the brain or vice versa (Meynert, Virchow, Lucas). The two hold 
a very close relation to one another. If premature stenosis sets in it is 
unquestionably sufficient to account for a small brain ; but, on the other hand, 
a growing brain evidently helps to expand a normal skull. The fact that de- 
fective brains are often covered by normal skulls if the defect has been 
compensated by a local accumulation of fluid, would seem to prove that as 
long as the same mechanical conditions are present the growth of the skull 
remains tolerably normal (Gudden). 

In order that we may be able to determine when a skull 
may be termed microcephalic, we must refer to the meas- 



596 THE NERVOUS DISEASES OF CHILDREN. 

urements of normal heads at certain ages (see Introductory 
Chapter). If a head which is supposed to be microcephalic 
fall far short of such average measurements the diagnosis 
of microcephalus may be safely made ; but the head may 
be developed in proportion to the rest of the body ; a small 
measurement in a child need not necessarily imply a con- 
dition of microcephalus, unless the measurement of the 
skull is below, while other measurements of the body are 
fully up to, the average in other children. 

The general measurements of the skull are not always sufficient to prove 
whether the brain is small or not. Each part of the skull should be inspected 
per se. Thus I have seen extremely small and receding foreheads with un- 
usually large occiputs. This would tend to give a measurement of a general 
circumference quite up to the standard, yet on closer inspection there would 
be no doubt that the frontal portions of the skull and of the brain were poorly 
developed, whereas the occipital portions were either normally developed or 
caused to bulge by the accumulation of hydrocephalic fluid within the ven- 
tricles. 

In several other heads which the author has examined this defect in the 
anterior portion was extremely marked, and as the frontal portion of the 
brain has least to do with motor and sensory functions, and more with the 
general intellectual development of the child, we can see the important bear- 
ing such facts have upon the prognosis of a child's future mental develop- 
ment. A frontal microcephalus may be present even though the cranial 
measurements be up to the average. 

The symptoms due to microcephalus are described 
under the heading of idiocy, and in the chapter on the Cere- 
bral Palsies of Children.* In this connection we may refer, 
however, to efforts made within recent years to correct 
these cranial deformities on the supposition that if the size of 
the cavity could be enlarged the brain would have a more 
favorable opportunity for further growth. Such reasoning 
could apply merely to those skulls in which the chief 
trouble lies in the premature synostosis of the sutures. If 
there is an inherent defect in the development of the brain, 
or if the brain growth has been checked as the result of ac- 
quired disease, such treatment is not applicable. The great 
difficulty, however, is, that it is not always easy to deter- 

* Of course microcephalic heads are at times found in persons of tolerable cerebral 
development. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. $97 

mine to which one of these causes the defective cerebral 
development is due. Lannelongue suggested a few years 
ago that in the case of microcephalic skulls a longitudinal 
opening be made in one or both halves of the skull, thus 
practically increasing the size of the intra-cranial cavity, or 
at least diminishing the pressure within this cavity. My 
experience has not been at all favorable to Lannelongue's 
doctrine, and I am willing to say that there is not the least 
reason to advise or encourage the operation, at least accord- 
ing to the method proposed by Lannelongue. This French 
surgeon operated upon a number of skulls, and claimed that 
after operation the children showed some signs of increased 
intelligence and were greatly benefited by the operation. 
Other surgeons have had but few favorable results to show, 
and of the cases operated upon there were not many in 
which a small brain was due to smallness of skull. I have 
had three children operated upon, and on one patient two 
operations were performed. Two patients with micro- 
cephalic skulls due to premature ossification of the sutures, 
died very shortly after the operation. The third case 
was a child, two years of age, whose skull had evidently 
undergone a premature synostosis. It survived the opera- 
tion and showed a little improvement, inasmuch as it began 
to play with other children in the hospital ward, but dur- 
ing the four months after operation did not learn either 
to talk, stand, or to walk. The improvement was, in fact, 
very slight indeed. Wishing to give the method a further 
test, the child was operated upon a second time, and an 
opening similar to the first was made in the opposite half of 
the skull. The child died from " shock " four hours after 
the operation was finished. 

Figure 156 is inserted to show the condition of the skull* 
after the second operation. It will be seen at first glance 
that the original opening that was made became very much 
smaller after four months ; that a tense fibrous tissue was 
formed over this opening, and that therefore four months 
after the initial operation the brain was not under condi- 
tions much more favorable than those which preceded the 

* This skull was demonstrated by Dr. A. Jacobi, at the last International Congress, 
in Rome, 1894. 



593 



THE NERVOUS DISEASES OF CHILDREN. 



first operation. I would therefore conclude from my own 
experience * that Lannelongue's operation is not likely per- 
manently to increase the intra-cranial capacity, and further- 
more, that operations upon young children are extremely 





Fig. 156. — Skull of a Child Two Years of Age, showing (on the left of the figure) the 
Narrowing of the Opening Made According to Lannelongue's Method Four Months 
Previous to Death. Death ensued four hours after second operation. 

dangerous, the children often dying from shock, or from 
the loss of blood. 

If we wish to utilize Lannelongue's idea it will be better 
to make a circular opening over that part of the brain 
which from the symptoms in the case would appear to be 
deficient. In children whose frontal lobes are stunted in 
growth, circular openings in the frontal or in the parietal 
bones would give a very large measure of relief, and I am 

* Bourneville and Jacobi have come to similar conclusions. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 599 

confident that if the operation is entirely aseptic cerebral 
hernia need not be feared. The bone should not be re- 
placed, but it will be well to provide such children with 
some form of external protection, or else the danger of di- 
rect injury to the brain from falls would be very great 
indeed. The author wishes once more to emphasize the 
fact that there are relatively few children in whom a small 
skull is the primary, or even the most important, cause of 
arrested development of the brain. The fault lies chiefly 
with the brain itself and cannot be remedied by operation. 

Partial Cerebral Defects. — In speaking of the con- 
dition of the brain in microcephalic children I have alluded 
to the fact that a part of the brain, and not necessarily the 
entire brain, may be checked in its development. This is 
particularly marked with regard to the frontal and the 
occipital lobes. These conditions deserve special atten- 
tion, moreover, because some of them are attended by 
symptoms which enable us to diagnosticate during life the 
exact area of defective development. It is fair to assume 
that if the frontal lobes are wanting, intelligence will suffer 
most, whereas the movements of the child will be normal, 
sensation will be undisturbed, and all the special senses, ex- 
cept possibly the olfactory sense, will be well developed. It 
is not claimed that intelligence is altogether the property of 
the frontal lobes, for the various functions that are so essen- 
tial to normal mental faculties are stored in the most diverse 
portions of the brain, and a defect in one or more of these 
sensory divisions of the brain in young children will natu- 
rally interfere with normal mental development. If the de- 
fect is in the motor areas, the disturbances of motion and of 
sensation will naturally point out the defective area. While 
porencephaly is extremely frequent in this region, I have 
not yet seen a single brain that exhibited normal develop- 
ment of its fissures and convolutions in all but the motor 
region. 

Defective Development of the Occipital Lobe. — 
Defective development of the occipital lobe, as indicated by 
an atrophy of the gyri of this lobe, is well illustrated by the 
case represented in Fig. 157, which has been reported by 
Peterson. " The patient was a female, twenty-eight years 



6oo 



THE NERVOUS DISEASES OF CHILDREN. 



of age, and was from birth an idiot, with epilepsy and left 
hemiplegia (slight) ; she was almost blind, being able only 
to distinguish light from darkness. Both occipital lobes 
were greatly atrophied ; the loss of tissue was made up for 
by the widely dilated posterior horns of the ventricles. 
The cerebellum projected far beyond the posterior lobes ; 




Fig. 157.* — Brain of a Woman Aged Twenty-eight years, Idiot from Birth and almost 
Blind. Defective development of both occipital lobes, which appear to be mere ap- 
pendages to the more normal anterior portions. Drawing made from specimen ; pia 
of left half not removed. 



the atrophy and sclerosis extended partly into the motor 
area of the right side." 

Defective development of the larger ganglia is not met 
with in cases in which there are no other large cerebral de- 
fects. This statement must be accepted with some reserve, 

* The author is indebted to Dr. Peterson for permission to have the specimen copied. 






DEFECTIVE DEVELOPMENT OF THE BRAIN. 601 

for little attention has been paid to this subject, and of the 
defective brains that have been examined the records say 
very little about the ganglionic region. 

The author has had occasion to diagnosticate defective 
development of the cerebellum. The diagnosis was made 
in a girl, eight years of age, born without difficulty and at 
full term. The child had never learned to walk or to talk. 
There was not a sign of spasticity or of actual paralysis. If 
the girl was placed on her feet she would fall over at once 
to the right. Equilibrium could not be maintained while 
the child was in the erect position. Sitting up in bed or on 
a table she was able to move the various parts of the body. 
The knee-jerks were absent ; hearing and sight normal. 
There was a condition of complete idiocy. The defect of 
the cerebellum was evidently part of a general brain defect. 
The case bore a superficial resemblance to Friedreich's dis- 
ease, but could be differentiated from it by the early onset 
and the development of idiocy very soon after birth. 

Agenesis Corticalis. — In the conditions previously 
described gross defects of the entire brain, or of parts of 
the brain, have been referred to. In the condition of agene- 
sis corticalis the size and weight of the brain may be en- 
tirely normal. The changes to be observed are those dis- 
covered on microscopical examination. The only outward 
indication of the imperfect development in the external 
configuration of the brain is abnormal fissuration. The 
fissure of Rolando dips straight into the fissure of Sylvius, 
without leaving a small convolution between the two fis- 
sures as in normal brains. (See Fig. 158.) The interparietal 
fissure extends farther back than normal into the occipital 
lobe; the island of Reil may be entirely exposed instead of 
being obscured from view by the margins of the fissure of 
Sylvius. Such differences as these are characteristic of a 
lower order of brain development (criminal brains, for in- 
stance). On minute examination of such brains that have 
been hardened, the large nerve-cells in the cortex are seen 
to be distorted, their contours not as well marked as in the 
normal brain, the protoplasmic contents are found in va- 
rious stages of disintegration. The nuclei are either sub- 
divided or destroyed, and the fine nerve - processes, so 



602 



THE NERVOUS DISEASES OF CHILDREN. 



beautifully distinct in the normal cells, have disappeared 
entirely. Sections through the cortical tissue of any part 
of the hemispheres reveal practically the same condition. 
The anatomical study of two cases, made some years ago, 
led to the recognition of a special type of idiocy with blind- 
ness and ending in marasmus. (See chapter on Hereditary 
Family Affections.) It is probable, however, that the agene- 
sis corticalis is not the sole morbid state in these special 
forms. 




Fig. 158. — Photograph of Brain of Author's First Case of the " Cerebral Type " of 
Hereditary Spastic Paralysis. The histological condition was described as an 
" agenesis corticalis." In the above figure, the confluence of fissures and the ex- 
posure of the Island of Reil are the signs of a low order of cerebral development. 
Through the hardening process the conditions have been accentuated a little, but 
they were present in the fresh specimen. At x, and in the frontal lobes, sections had 
been removed for histological examination. Other letters refer to fissures. 



MACROCEPHALUS. 

This term may be applied equally well to an increase in the size of the 
head due to hydrocephalus, as to those rarer conditions in which an in- 
crease in the volume of the brain has brought about a corresponding in- 
crease in that of the skull. Hypertrophy of the brain is very rare indeed. 
While some maintain that the increased size of the brain is due to a prolif- 
eration of the neuroglia, others believe that it is the result of an increase 
in all the elementary structures of the brain, but the histological examination 
has been made in so few cases that the point has not been decided. Steffen 






DEFECTIVE DEVELOPMENT OF THE BRAIN. 603 

remarks correctly enough that if the increased size were due to a prolifera- 
tion of the neuroglia, this proliferation would come to a standstill and a par- 
tial atrophy and sclerosis would be the natural result, but in those brains 
which have been examined post mortem true hypertrophy was found and no 
indications of a sclerotic condition. Rilliet and Barthez, Gerhardt and others, 
evidently confound cases of interstitial encephalitis with hypertrophy of the 
brain. Steffen is correct in insisting that the term should be restricted to 
those in which there is a true hypertrophy of a part of, or of the whole brain. 
In consequence of the increased growth of the brain the resisting skull 
causes increased pressure ; as a result of this the blood-vessels are narrowed 
and the circulation is impaired, whence it follows that the brain is pale and 
anaemic ; the convolutions are often considerably flattened, and the fissures 
become more or less indistinct. The ventricles may be greatly compressed, 
or almost obliterated. By compression from without the cerebro-spinal fluid 
is driven largely out of the ventricles. As long as the fontanelles remain open 
the danger from compression is not as great as it is in later years when the 
entire skull represents an unyielding mass. As long as the fontanelles re- 
main open a true hypertrophy can be differentiated from a chronic hydro- 
cephalus. The hydrocephalus increases more rapidly as a rule than the 
hypertrophy does. The fontanelle will be more distinctly pulsating in hydro- 
cephalus than in the other condition. The form of the head will vary much 
in these two processes. In the case of an hypertrophied brain the entire 
skull will be equally enlarged ; whereas in hydrocephalus the accumulation 
of fluid in the anterior or the posterior horns of the ventricles tends to a special 
increase in the frontal and in the occipital portion of the skull. The sutures 
may be forced apart by the increase in fluid. 

The clinical symptoms of this condition are not very different from those 
found in chronic hydrocephalus ; a considerable mental development, or even 
normal mental development, is compatible with hypertrophy of the brain 
tissue, and if the fontanelles are not closed the increase in the size of the 
brain does not entail a very considerable increase of pressure, but it is false 
to suppose that children with hypertrophied brains would naturally be 
brighter than those whose brains are of normal dimensions. The hyper- 
trophy is self-limited by reason of the additional pressure it causes. 

If the size of the head increases very much, children are unable to carry 
the head, feel the need of supporting it, become apathetic, morose, and have 
a tendency to fall ; if the anaemia increases, convulsions, disturbances of vi- 
sion, vomiting, and a gradual cessation of all the faculties may precede death, 
which occurs after an indefinite period of time, unless brought about more 
rapidly by an intercurrent disease. It will not do to devote too much atten- 
tion to this condition, which is of extremely little practical value. The diag- 
nosis of hypertrophied brain will be made much more frequently at the post- 
mortem table than in the wards of a hospital or in private practice. 

Defective Development of the Cranial Nerve 
Nuclei should be considered in conjunction with the larger 



604 THE NERVOUS DISEASES OF CHILDREN. 

defects described above. These cases have received but 
very little attention, yet they are of great interest both 
from a clinical and from an anatomical stand-point. The 
nuclei most frequently affected are those connected with the 
nerves governing the ocular muscles. Cases of congenital 
ptosis, unilateral or bilateral, are so common that every 
one can recall persons so affected. Forms of congenital 
deficiencies in facial innervation come under this heading. 
Gowers has made a short note of them under the heading 
of " Infantile Oculo-facial Palsy." Moebius collected over 
forty cases of this class and reported upon them in the year 
1892 ; while Schapringer gave a most intelligent descrip- 
tion of a similar condition which he termed " Congenital 
Bilateral neuroplegia (Paralysis of Lateral Movements) 
and Facial Palsy." His case, which we shall give some- 
what more in detail, shows, however, that his title did not 
exhaust the clinical symptoms of the case, for there was evi- 
dence of involvement of the fifth nerve as well as of the sixth 
and seventh. I should, therefore, prefer to give to all these 
diseases the proper designation of " Congenital Nuclear 
Palsy." That there is in these conditions an actual defect 
in the development of the cranial nuclei there can be little 
doubt, and that these defects are associated with other con- 
ditions of defective development, or of primary degenera- 
tion, is proved by the occurrence of these congenital nu- 
clear palsies with imbecility or idiocy. 

I have seen several interesting cases of congenital 
nuclear palsy in patients afflicted with scleroderma and 
myxcedema. We shall be justified, therefore, in relegating 
to this class of cases all those patients in whom there is a 
congenital defect of a unilateral or bilateral character im- 
plying insufficient innervation of the muscles governed by 
any of the cranial nerves. The forms that are most fre- 
quently observed are congenital ptosis, ophthalmoplegia 
externa, partial or complete, facial paralysis, and paralysis 
of the tongue. A close clinical and anatomical relationship 
would seem to me to exist between these cases of congenital 
nuclear palsy, and cases of hereditary progressive muscular 
atrophy, possibly also between these nuclear palsies and 
those with local muscular defects. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 



605 



The symptoms of these types will naturally vary very much. I propose 
giving a short summary of Schapringer's case, which exhibits a more complex 
association of symptoms than any other with which I have become acquainted. 
The history in brief is this : A girl, eight years of age (Fig. 159), who was 
suffering at the time from a slight bronchial trouble, was examined by Dr. 
Schapringer in 1889. The child was of normal development, and of average 
intelligence, although she was not sent to school, and had not yet learned 
to read. In walking she stumbled and fell more frequently than other chil- 
dren of the same age. She states that this is in consequence of a weakness 
in the legs, and not on account of the visual disturbance. Her face is pale, 
expressionless, and mask-like. The naso-labial fold is absent on both sides, 
there is not the slightest indication of 
folds in the integument of the face 
either in laughing or crying. Her 
laughing and crying sounds cannot 
easily be distinguished. She cannot 
close her lips. Substitutes Unguals for 
labials, " tata " for " papa," etc. The 
right angle of the mouth is drawn 
downward and outward. Whistling, 
blowing, etc., are impossible. She is 
not able to wrinkle the forehead either 
vertically or transversely. When her 
eyes are open the ordinary amount of 
eyeball is uncovered. Quite often one 
lid hangs a little lower than its fellow, 
but the eyes can be fully and com- 
pletely opened, proving that both leva- 
tores palpebrarum are not involved. 
Complete closure of the eyes is impos- 
sible, although the lids can upon forci- 
ble effort be closely approximated to 

one another. There is a distinct epicanthus, the caruncle and plica semilu- 
naris are only slightly developed on both sides. 

Ordinarily the axes of the eyes are parallel, frequently, however, the right 
eye is directed a little more upward and slightly outward. She is able to 
follow an object with both eyes if it be lowered or elevated in the median 
line. If the object be approached to her face, keeping it in the saggital plane 
she follows it readily, and she will keep her eyes fixed upon a finger held 
near the nose for a long period without fatigue. These, however, are all the 
movements of which the eyeball is capable. The two internal recti contract 
freely if required in order to bring the eyes into convergence for near objects. 
If, however, one of these muscles be required to act in unison with the ex- 
ternal rectus of the opposite eye for a conjugate lateral vision, there will be 
found an utter inability to do so. The two external recti muscles are either 
completely paralyzed or very deficient. The internal recti are not wholly 
paralyzed. When the child wishes to see an object situated to the side 




Fig. 159.— Girl, Eight Years Old, with 
Congenital Pleuroplegia. ( Scha- 
pringer.) 



606 THE NERVOUS DISEASES OF CHILDREN. 

of the median plane, instead of rotating the eyes she turns her entire head. 
The fundus appears normal, except that the vessels are a little more 
tortuous than usual. The functions of the ciliary muscles are normal. 
There is no strabismus convergens. The size and mobility of the pupils 
are normal. When the tongue is protruded it appears to the left of the me- 
dian line ; the left half is a trifle smaller than the right. When eating, the 
child is obliged to use its finger to dislodge food from the cheeks. She is 
unable to masticate hard substances, such as crusts. She can move the 
lower jaw laterally toward the right side, but not toward the left ; thus indi- 
cating a paralysis of the right pterygoid muscle. She has uvula bifida. There 
is an hypertrophied condition of the canine ligament of the right side, and 
normal development of the left. In addition to these congenital defects the 
author mentions a deflection of the distal phalanges of the index-fingers at an 
angle of about one hundred and fifty degrees in the direction of the middle 
finger. He interprets this also to be a congenital abnormality. The child has 
the condition known as " funnel chest." 

Reviewing the symptoms, which I have quoted very 
freely from Dr. Schapringer's paper, it is evident that the 
congenital defects involve the motor branch of the fifth, the 
hypoglossal, and the facial nerve, also the tracts which gov- 
ern the conjugate lateral movements, viz., the third and 
sixth nerve nuclei, and the nerve-tracts connecting the 
same. The only criticism to be passed upon this interest- 
ing case of Dr. Schapringer's is that while he has invented 
a good term for the paralysis of the lateral movements, 
the case presents other symptoms; and that the congeni- 
tal bilateral pleuroplegia is the most prominent symptom 
of the condition which we might define in an impartial 
way as one of bilateral congenital nuclear palsy. Cases of 
this description are evidently rare, for Schapringer had 
been able to collect but four others like the one he de- 
scribed. 

During the past few years a number of authors have 
reported instances of congenital unilateral facial palsy, pre- 
sumably due to defective development of the nucleus. 
Schultze described this condition in a girl of five years, 
Bernhardt in a man of twenty-four years; and Remak has 
given an account of a young man of eighteen years, who 
presented a congenital defect of the platysma myoides, as- 
sociated with slight bilateral ptosis, and a limitation of 
ocular movements upward. 



DEFECTIVE DEVELOPMENT OF THE BRAIN. 60J 

The treatment of these disorders is to be intrusted to 
the surgical skill of the oculist. 

BIBLIOGRAPHY. 

Anton: Ueber angeborene Erkrankungen, etc. Vienna, 1890. 

Bernhardt: Neurologisches Centralblatt, 1894, No. 1. 

Binswanger : Virch. Arch., vol. xci. 

Bourneville : Recherches sur l'Epilepsie, etc. Paris, 1894. 

Chisolm : Archives of Ophthalmology, vol. xi., p. 323. 

Gowers : Manual, Am. Ed., vol. ii., p. 197. 

Graefe : Handbuch d. Augenhk., vol. vi., p. 60. 

Harlan: Transactions of the American Ophthalmological Society, 1881, p. 

216. (The last three authors quoted after Schapringer.) 
Jacobi : " Non Nocerl," New York Medical Record, May 19, 1894. 
Kundrat: Die Porencephalic. Graz, 1882. 
Lannelongue : Noveau Iconog. de la Salpetriere, 1891, p. 89. 
Moebius: Miinchener Med. Wochenschrift, 1888, No. 6; 1892, No. 2-4. 
Peterson: Proceedings of the New York Pathological Society, 1894, pp. 94, 

98. 
Remak: Neurolog. Centralbl., 1894, No. 7. 

Schapringer: New Yorker Medizinische Monatsschrift, December, 1889. 
Schultze : Porencephalie. Heidelberg, 1886; Neurolog. Centralbl., 1892, 

No. 14. 
Steffen : In Gerhardt's Handbuch, vol. v., 209 et seq. 



DISEASES OF THE MIND. 
CHAPTER XXXII. 

INSANITY. 

In the preceding chapters we have discussed the or- 
ganic diseases of the brain, and have touched upon con- 
ditions due to arrest of development and to morbid affec- 
tions of the central nervous system during the period of early 
growth. Mental disorders may be similarly subdivided. 
Idiocy represents the psychic derangement due to arrested 
development; it is so distinct and so large a subject that it 
will be treated separately. Insanity includes the morbid 
conditions coming on after a fair degree of mental develop- 
ment has been reached, and constitutes the subject-matter 
of this chapter. 

Insanity in the child resembles closely that in the adult. Such differ- 
ences as do exist are dependent upon the unusual sensitiveness and irritabil- 
ity of the youthful mind, and upon the paucity of concepts which have been 
formed prior to the development of the insanity. At a very early day sensory 
impressions become fixed ; hallucinations are possible, therefore, in very young 
children. But the normal mind does not develop systematized concepts nor 
the logical faculty during the first few years of life, and prior to this stage of 
mental development delusions — faulty, unfounded beliefs — cannot exist. The 
delusions of an insane child will differ from those of the adult in view of the 
limited ideas it possessed during the period of mental health. Its delusions 
will be concerned largely with its own individuality and its relation to the 
family, its teacher, and its God ; but the delusions of the adult in regard to 
his wealth, his position in the religious, social, and political world, are entirely 
foreign to the child's mind. 

The normal child lacks, moreover, the judgment and the power of self- 
restraint by which the adult is guided and controlled in his actions. Mey' 
nert has charmingly depicted the egotism of the child, and the constant 
struggle of the healthy individual to rid himself of the ideas of self until he 



INSANITY. 609 

is enabled to sacrifice his individuality and his entire being for the good of 
the family, the state, and of all mankind. The child for a long time remains 
upon that lower plane in which passion and the gratification of desires are 
the mainspring of all action. 

The experience of centuries has taught us that by gradual stages, and 
through the influence of training and education, the selfish views of the 
child can be transformed into the more generous views of the adult. This 
process of evolution may be checked by disease ; it is only by comparison 
with the views and attainments of children of the same age that we can es- 
tablish the standard to which every child should conform. 

Insanity in childhood is relatively rare. According to 
the statistics of Hagen (quoted by Spitzka) only one in 
70,684 children annually becomes insane, excluding those 
born so. Spitzka states that " over four per cent, of 3,244 
adult recorded private cases may be justly regarded as 
having been insane in childhood, while but twelve patients 
afflicted with infantile forms of insanity " were observed by 
him during their infancy. From this it is evident that we 
must not only regard the full-fledged insanities of early 
life, but also those morbid conditions in early years which 
foreshadow the outbreak of mental disease in later years. 

The psychoses are not developed in very young infants, 
although Greding reports a child that was a " raving 
maniac " at birth. The story must be taken with a grain of 
salt. Rush has noted the occurrence of mania at the age of 
two years, and Sinkler has observed it in children three 
years old. 

After the period of first dentition is past, faulty psychic 
inheritance may become noticeable ; but the age of puberty 
is the one fraught with greatest danger. The author had 
under observation various members of a family, all of whom 
(two boys and three girls) have developed mental disease 
between the ages of twelve and fifteen years. All classes 
of the social body seem to be equally prone to mental dis- 
ease ; while idiocy is unquestionably more frequent in the 
poorer classes than among the well-to-do. 

Etiology. — Heredity is the potent factor in the cau- 
sation of juvenile and adult insanity. There is always the 
danger of over-estimating the importance of any one factor. 
but the daily experience of alienists and neurologists helps 
to enforce this belief. The importance of heredity is im- 
39 



6 10 THE NERVOUS DISEASES OF CHILDREN. 

pressed upon us, too, by the different course of acquired 
insanity in persons with or without an hereditary taint. 
Psychic disorders constitute, as a rule, the last in a series 
of degenerative diseases ; neurasthenia, chorea, epilepsy in 
the parent may lead to insanity or idiocy in the child. By 
way of exception healthy individuals may emanate from 
families with a distinct neurotic or psychic taint. Mental 
derangement in children is ordinarily developed, if one or 
both parents have been insane, if one or both have been 
afflicted with a severe neurosis, if one or both have been 
addicted to alcohol, and above all, if the parents are related, 
and belong to a family in which nervous or mental disease 
has been of common occurrence. Individuals of different 
but tainted families will also be liable to generate insane 
children, or such as show distinct stigmata of degeneration 
(deformities of the skull, of the ears, gothic palate, stunted 
growth, abnormal sexual appetite, early masturbation, and 
the like). Closely allied to the hereditary forms of juvenile 
insanity are those due to traumatism during birth. Meyer 
has insisted on this. The author has seen innumerable 
cases of idiocy due to this cause, but not a single instance 
of any other form of mental derangement. 

Arrested physical growth is often associated with men- 
tal impairment due to hereditary causes. A girl, aged 
twenty, Avhom I have seen in repeated maniacal attacks, 
is stunted in growth, being only as tall as a child of 
twelve, without any sign of mammary development or of 
menstruation. The girl stutters a little, but is fairly bright 
in the interval between the maniacal attacks. 

Among the causes of insanity acquired in early child- 
hood, the chief ones are traumatic injuries to the skull. Em- 
minghaus has found this origin in fourteen of one hundred 
and three cases, not including those in which the injury was 
followed by epilepsy and mental derangement. Loss of 
memory and complete dementia, or maniacal excitement, 
are the forms of insanity commonly developed from this 
cause. The occurrence of idiocy after relatively slight in- 
juries need not be insisted on in this connection. Menin- 
gitis early in life, ear disease, insolation, epilepsy, hysteria, 
chorea, severe emotional excitement, grief, shame, disease of 






INSANITY. 6ll 

the heart, intestinal parasites, acute infectious diseases are 
the conditions which predispose to the development of in- 
sanity. To these might be added excessive work at school, 
maltreatment at home, starvation, and cruel punishments. 

Masturbation is another and a potent cause. The impor- 
tance of this factor has been underrated by some ; it can 
scarcely be overrated. There is a just difference of opinion, 
however, as to the influence it exerts in generating mental 
disease. Many claim that it is often the early symptom of 
an insane condition. There can be no doubt of this, but it 
is equally certain that the continuance of the habit brings 
about a rapid deterioration of the individual's mental state. 
It is often acquired by imitation, and quite as often it would 
seem to be the result of natural tendencies. In the former 
class the practice may occasionally be checked ; in the lat- 
ter, the habit is continued, both in boys and girls, in spite 
of the most persistent watching. The more stubborn cases 
are generally those characterized by other distinct signs of 
degeneration. The evil effects of the vice become most 
pronounced at the time when the individual is subjected to 
the first serious strain. Several of such young patients 
have done well at school, have passed their examinations 
successfully, but have broken down completely in the firrt 
competition with boys of mature and normal intellects. 

FORMS OF INSANITY. 

A detailed account of all the symptoms of insanity 
would far exceed the limits of this treatise. By combining 
a discussion of the symptoms with a description of the types 
of mental derangement, as it occurs in early life, we shall 
be able to give a sufficient survey of the entire subject. It 
is best to begin with those symptoms and conditions which 
are on the border-line between sanity and insanity. 

Imperative Concepts. — The orderly sequence of 
thought in the child and in the adult may be interrupted 
by foreign and recurrent concepts, which obtrude them- 
selves upon the individual's consciousness, which annoy him 
and tyrannize over him, but of which he is not able to rid 
himself. 



6l2 THE NERVOUS DISEASES OF CHILDREN. 

These imperative or insistent concepts are very common in 
childhood ; at times they disappear without leaving any 
trace of mental defect behind them ; in some children they 
represent the first stages of chronic forms of insanity. 
Imperative conceptions may lead to imperative actions, to 
morbid fears, and morbid impulses. 

Many normal persons have passed through physiological states akin to 
these imperative concepts. On going to bed the thought arises suddenly 
that the light has not been properly turned down, that the gas might be 
escaping. They assure themselves that all is well, and after a few minutes 
the same fear or concept comes upon them again. The person so troubled 
may attempt to allay his fears by methods which ordinarily carry conviction, 
but in spite of himself the same insistent idea annoys him. Returning to 
one's door again and again to make sure that it is properly locked ; the an- 
noying thought that the letter which has just been mailed was not properly 
signed or sealed ; such are some of the imperative concepts of daily life. 
The sight of a knife may suggest the harm that can be done with it ; a per- 
son standing on a precipice or on a high tower has a feeling as though he 
would be dragged into the depths below. The child, after it has passed the 
first five or six years of life, may have similar experiences. It is often tort- 
ured by these insistent concepts. Some pupils at school have to do every- 
thing over and over again ; they never finish a sum in arithmetic as quickly as 
others because the doubt arises whether they have done it properly, and they 
are impelled to do everything a definite number of times (arithmomania). As 
a rule, they can assign no reason for this. A little patient would never ad- 
dress any one without repeating the name twice ; he was conscious of this 
peculiarity, was finally induced to repeat the name a second time to himself 
in such a low tone of voice that no one could hear it, and gradually lost the 
habit. 

Another lad, well known to the author, would do everything three times 
for fear of having done it twice. He would take three steps at a time in going 
upstairs ; if there were but two he would jump back from the first and then 
walk up the two, so as to make three in all ; before going to bed he touched 
the floor three times ; as soon as he was in bed he would get up again to 
make sure that he did not touch it twice. These concepts and actions were 
imperative, but they were developed from a fear that was engendered in him 
at the time of his father's death that his mother might be the second one to 
die. This caused him to avoid " two " in every way. After a number of 
years the imperative concepts were overcome. 

Closely allied to these conditions are the fears of con- 
tamination (the mysophobia of Hammond ; delire du 
toucher of Legrand du Saulle). I have known several 
children who would not touch the knob of a door, or take 



INSANITY. 613 

anything out of other people's hands. One child would 
not be washed because the water flowed through pipes and 
the pipes were not clean. The fear of open places (agora- 
phobia of Westphal) occurs in children, though not nearly 
so often as in adults. Some years ago the author treated a 
young girl, twelve years of age, who could not be induced 
to come to his office because she would have to cross a 
small hill over a tunnel ; she was certain that she could 
never get across. These fears may increase in such num- 
ber as to give rise to what Mills calls pantophobia. 

Children as well as adults are troubled by insistent ques- 
tions — generally abstract ones — which interrupt their trains 
of thought. Why is the sky blue, the water wet, the ball 
round ? Occasionally they soar into the field of religion. 
Why is God just? and so on. 

All these conditions may be of slight significance unless 
they lead to imperative acts of violence (cutting a child's 
throat at the sight of a knife, etc.) ; or unless the effort to 
suppress these morbid concepts and impulses produces great 
excitement. A little patient of mine, years ago, was so 
annoyed that he would every now and then pass into a 
maniacal state or entertain suicidal ideas. Westphal was of 
the opinion that these imperative conceptions were never 
developed into delusions ; this is true in general, but they 
are often present in persons who develop systematized 
delusions suggested by the contents of some one imperative 
conception. Imperative concepts and impulses are very 
apt to occur in children who have passed through some ex- 
hausting disease, or some intense emotional excitement. As 
children grow older they may learn to disregard annoying 
concepts, and thus rid themselves of them. 

Cerebral Neurasthenia. — Nervous exhaustion, or 
neurasthenia, is, on the whole, so rare in children that we 
have not devoted any special chapter to the discussion of 
this disease. The causes which give rise to neurasthenia in 
the adult are far less potent in childhood. Excitement, 
over-work, cares in early years, are more apt to produce 
hysterical and choreic conditions than neurasthenia ; occa- 
sionally, however, the cerebral tvpe is developed in young 
persons between the ages of ten and fifteen years or older. 



6 14 THE NERVOUS DISEASES OF CHILDREN. 

The chief symptoms are, inability to concentrate the atten- 
tion upon any special work, or excessive restlessness follow- 
ing upon such an effort. A feeling of pressure upon the 
top of the head, a dragging sensation over the nape of the 
neck and the upper portion of the spine, general irritability, 
a morose disposition, sleeplessness and a feeling of fatigue, 
a slight increase in the reflexes, sensations of heat and cold 
complete the details of this condition. 

Cerebral neurasthenia occurs mainly in the children of 
hysterical or excessively neurotic parents, and in those who 
come of healthy parentage but are pushed inordinately in 
their work at school to satisfy the demands of ambitious 
mothers. It has been the custom to attach the entire blame 
to present educational methods, but the chief fault lies with 
the father or mother who cannot recognize the child's in- 
ability to cope with other children of the same age. Boys 
or girls who recognize that they are at a disadvantage in 
competition with others become discouraged and moody, 
and often develop a condition of melancholy depression. 



Not long since, a young girl, aged thirteen, was brought to me, who had 
just entered upon the work of a high-school in which an older sister had 
graduated with high honors. The patient was told by her mother that she 
would be a discredit to her family unless she did at least as well as the older 
sister had done. The child made a strenuous effort to come up to these 
expectations, but the result was evident on examination. The child com- 
plained of intense headaches, frontal and vertical, of a feeling as though 
she were being crushed under a weight. She looked pale and haggard 
in consequence of the loss of sleep. Her appetite had left her, and for a few 
days before she was examined was either in a drowsy condition or else 
would cry and complain of her misfortune. She knew that she could not 
possibly do satisfactory work at school, and had developed the idea that she 
was thoroughly unworthy of the care which her parents had bestowed upon 
her. 

The child was taken out of school at once, sent to some relatives in the 
country, and was told that she would get entirely well provided she would 
not think of school, or anything connected with school matters, for a period of 
several months. The parents were also instructed never again to force the 
child's work, or to foster any inordinate ambition in her. The older sister 
was a bright, strong, well-developed girl, who could in all probability have 
stood any amount of work without showing signs of fatigue. The younger 
child was less well developed physically, and presented the remnants of 
former internal hydrocephalus (a bulging forehead and very large transverse 



INSANITY. 6l$ 

occipital diameter). Had the parents been allowed to continue in their foolish 
course much longer, this child, who presented on the whole none but neuras- 
thenic symptoms, would unquestionably have developed a serious form of 
psychosis. 

The course of cerebral neurasthenia may cover a period 
of several weeks or months, according to the time at which 
a radical effort is begun to check the development of the 
disease ; but the affection is an eminently curable one. The 
condition should be treated very much as is the same disease 
in the adult. The most important point is to give body and 
mind complete rest, to take the child from school at once, 
prohibit all mental fatigue either in the way of school or of 
home studies. If a proper arrangement can be made, it 
is best to place the child under care of a sensible nurse, and 
subject it to the rest-cure. A separation from hysterical 
and irritating relatives is absolutely essential. Careful feed- 
ing and the ordinary tonics — above all, iron, arsenic, and 
small doses of strychnia — will help to bring about a rapid 
improvement. After four to six weeks the child should be 
allowed to roam about in the country, and its mind should 
at least for a year or more be kept entirely free from every 
sense of duty and obligation. 

Hypochondriasis is often associated with neurasthenia, 
and occasionally it is a primary affection. The fear of in- 
sanity is not as pronounced as in adult neurasthenics, but 
the fear of death is common. The uncomfortable sensations 
in the head frighten the child, and soon it takes notice of 
every little symptom to Avhich other children would pay 
no attention. In such introspection children are encour- 
aged by over-anxious mothers. Like the adult, the young 
hypochondriac hears his heart palpitate, takes notice of the 
rumbling of his bowels, watches his skin most carefully, 
and exaggerates the importance of every pimple that ap- 
pears upon it. At the age of puberty a boy's attention is 
rivetted easily upon the sexual organs. He may be fright- 
ened by the occurrence of erections or by the difference in 
the size of the testicles. A bright lad, aged fourteen, whom 
I treated for a long time, and who came of a highly neu- 
rotic family, was at great pains to prove to me that his 
testicles were "detached" from the rest of his bodv.be- 



6l6 THE NERVOUS DISEASES OF CHILDREN. 

cause he could move them about freely. Early masturbation 
intensifies such fears. Westphal reports the history of a 
young hypochondriac who complained of abnormal sen- 
sations in the head, in the feet, in the abdomen ; his tears 
had been dried up, and all the mucus in the lungs had been 
expectorated. The boy's hypochondriacal mood was inter- 
rupted by a mild attack of maniacal excitement. The prog- 
nosis of hypochondriasis in the young is entirely favorable 
unless it is based upon a marked hereditary taint. The cure 
of the condition lies in sensible management of the youth's 
mode of life, in diverting his mind from himself by moder- 
ate indulgence in out-door sports, by providing him with 
the companionship of sober-minded lads of the same age. 
I have known boys suffering from sexual hypochondriasis 
to be forced by parents into early intercourse with the 
opposite sex. The procedure is invariably harmful. 

The preceding forms of mental derangement are less 
serious than those which we must now consider. 

Mania. — The term " mania " denotes a form of mental 
disease in which there is a marked acceleration of all cere- 
bral and physical functions, generally associated with a 
feeling of well-being. In children this condition is rela- 
tively rare ; it is often confounded with a temporary active 
delirium, which comes on after the ordinary febrile diseases 
of childhood. In true mania restraint is entirely removed ; 
the patient's ability and desire to do anything and every- 
thing he chooses knows no bounds. Thoughts and impulses 
follow rapidly upon one another. Emminghaus has well 
said that an exalted mood, a rapid succession of incomplete 
thoughts, unbounded desire to make everything it sees its 
own, are characteristic of the healthy child. But the normal 
child, under the restraining influences of education and train- 
ing, soon learns to curb its desires and to develop an orderly 
train of thought. The young maniac has apparently cast 
aside all restraint and gives himself up completely to his 
passions and his impulses. His incessant activity leads him 
to tear and destroy everything within his reach ; he is cruel 
to others and does not hesitate to inflict injury upon him- 
self. There is no trace of a sense of decency or propriety, 
even young children using the foulest language. Immature 



INSANITY. 617 

thoughts follow rapidly upon one another ; everything the 
maniac hears amuses him ; a word suggests another that 
rhymes with it ; his answers are often quick, sometimes 
bright ; but they are flashes merely and do not denote real 
intelligence. 

The excessive restlessness and the rapid flight of ideas 
are not followed by a feeling of fatigue, which would be 
natural in a normal person. Loss of appetite and of sleep 
contribute to the exhaustion which is developed if the con- 
dition lasts for a number of weeks or months. 

The symptoms of mania come on in an insidious 
fashion, or may be preceded by a period of depression. At 
first the greater liveliness and activity of the child are sup- 
posed to be a mere exaggeration of its normal state ; by 
degrees the increase of all the symptoms points to a morbid 
state ; after the acme of the disease has been reached the 
condition remains unchanged for some weeks, and then the 
excitement gradually diminishes. A return of sleep is, as a 
rule, the first favorable sign pointing toward recovery. Re- 
current mania (several attacks and remissions) has been 
described. The exact duration of a maniacal attack may 
vary between five months and a year, but the state of in- 
tense excitement is, as a rule, much shorter. 

Among the etiological factors the acute febrile condi- 
tions and severe strain or emotional excitement are the 
most potent. The period of beginning menstruation is 
fraught with the greatest danger. The only cases of true 
mania which I have seen were in girls at this period, and in 
a boy, aged fifteen. One of the girls had passed through 
a series of examinations at school with great credit to her- 
self. On the morning following the last examination she 
refused to get out of bed, would not take her food, and 
would not allow herself to be washed. To all questions why 
she would not do as asked, she mumbled a few inaudible 
words ; soon she became wholly silent, and for a period of four 
weeks she lay in an absolutely stuporous condition, passing 
urine and faeces into the bed, and refusing to take nourish- 
ment. From this condition she passed quite suddenly into 
one of most violent mania, in which she tore everything 
that she could lay hands on, sang and cursed all day long, 



6l8 THE NERVOUS DISEASES OF CHILDREN. 

spat at every one, tore her own clothes into shreds, and 
would expose her person before every one. (In health she 
was a sweet well-behaved child.) The state of maniacal 
excitement lasted four months, during which time she lost a 
great deal of flesh. Six months after the first symptoms had 
appeared, menstruation set in, and from that time onward 
a rapid and complete recovery ensued. In other cases I 
have observed a development of mania after the cessation 
of the menses that had appeared a few times. A close 
relation between the menstrual flow and these psychic con- 
ditions cannot be doubted. 

While the prognosis is favorable, careful treatment is 
necessary in all cases. These children must be guarded by 
competent nurses ; mechanical restraint cannot always be 
avoided, but it should not be more forcible than necessary. 
Such patients are generally fit subjects for an asylum, and 
often do better there than at home, where greater restraint 
has to be applied. The author has treated such patients, 
however, in their homes by placing them in large, well-venti- 
lated rooms, away from the rest of the family and under 
the charge of two or more nurses. 

In home and asylum treatment, the hydrobromate of 
hyoscin (one two-hundredth to one one-hundredth grain 
three times daily), given by the mouth or hypodermically, 
is a valuable drug. In addition, sulfonal or trional, in ten 
to twenty-grain doses, should be given at night. Bromides 
are entirely useless, and opiates of little benefit. Prolonged 
full baths, followed by slightly cooler ablutions of the 
spine tend to calm the excitement, and sometimes help 
to induce sleep when all other measures fail. 

Melancholia represents a distinct form of insanity 
which has been observed in children, particularly between 
the ages of eight and fifteen years. It is not to be con- 
founded with simple melancholy depression, which may 
accompany many other morbid mental states. It is as nat- 
ural for a lunatic who supposes himself to be the victim of 
persecution, or a target for the raillery of others, to be de- 
pressed and melancholy as it would be for a sane person 
who found that all his efforts to succeed were in vain, and 
that everything and everybody were against him. 



INSANITY. 619 

In true melancholia the child is depressed without 
cause. Experiences which would please others make no 
impression upon its saddened mind. It does not care to 
play with other children, whose frolicsome ways are a 
source of annoyance. Games, books, theatres, have no 
charm for the melancholy child ; it seeks seclusion, sits in 
a corner by itself all day long ; will not speak spontane- 
ously, and, if spoken to, either does not answer at all or 
replies in monosyllables and with much hesitation. No 
reason for its moodiness is given ; after much questioning 
it may say that it feels sad, but does not know why. To 
the questions put to a little patient I received the answer, 
" I know, I know," accompanied by a nodding of the head. 
As it was recovering, the child stated that she knew every- 
one was against her; that her parents did not care for her; 
that she was to be punished because she had not loved 
them enough, and because she was so ugly (as a nlatter of 
fact she was good-looking). As this same child was taken 
to the country to hasten convalescence, she said : " I know, 
I know, you will burn me to ashes." 

In contradiction to the acceleration of all mental proc- 
esses in mania there is a distinct inhibition, a " slowing 
up " of all mental and physical processes in melancholia. 
Loss of appetite, restless sleep, slowness of all muscular 
efforts, obstinate constipation are characteristic features of 
this disorder. The child may be able to reason a little re- 
garding its unfortunate condition, and self-accusations are 
abundant. It accuses itself of unkindness toward others, 
of want of respect and love for its elders ; if it has had any 
religious training it develops the ideas of the " unpardon- 
able sin," and of lack of devotion to God — ideas which 
play a prominent part in the melancholy of adults. Hallu- 
cinations in keeping with the depressed mood, the sight 
of the devil, of a cruel teacher armed with all sorts of 
weapons of torture, may increase the depression, or else 
lead to a condition of excitement or frenzy (melancholia 
agitata). Unlike the maniac, the melancholy patient di- 
rects his frenzied impulses toward his own person. Self- 
mutilation is common. A young girl, aged thirteen, had 
to be restrained because she would insist upon tear- 



620 THE NERVOUS DISEASES OF CHILDREN. 






ing out her hair and attempted to cut deep into her 
skin. 

Suicide of children is frequently the result of melancholia. Like the 
adult, the child seeks to put an end to a life that is so full of trouble and mis- 
ery. Statistics as to the frequency of suicide in children are rather unsatis- 
factory ; Emminghaus quotes those of Morselli that show the number to be 
greatest in Denmark and Prussia, and smallest in Belgium and Italy. The 
author has seen no statistics bearing upon America. In the majority of 
cases some mental disease is the cause of suicide in the young, and none is 
more frequent than melancholy. Some suicides are due to trifling causes 
— fear of punishment, chagrin over an unmerited rebuke, etc. 

Melancholia with stupor (melancholia attonita) is a more 
extreme form, which is either developed primarily or fol- 
lows upon a condition of simple melancholy. In this form 
the child lies in bed absolutely motionless, takes no notice 
of its surroundings, and cannnot be induced to smile or to 
say a single word. It reacts but feebly to every form of 
cutaneous stimulation ; it will to a certain degree tolerate 
pain rather than move. If passive movement is attempted, 
the limbs will retain the position given them, or the child 
offers considerable resistance. Urine and fasces are passed 
into the bed. During this condition of stupor disagreeable 
hallucinations and terrorizing delusions add to the child's 
misery. 

In the author's experience melancholia is the most fre- 
quent form of mental derangement in childhood. Its 
course varies very much. Some patients get well in a few 
months, others do not recover for more than a year. In a 
number of instances a condition of mania follows upon the 
period of depression. The prognosis is favorable as regards 
ultimate recovery ; it is certain that sixty per cent, of 
young melancholy subjects get well. As a rule, the pros- 
pects of early recovery are better in the agitated form, and 
less bright in the stuporous form. Melancholy may ter- 
minate in dementia, or in death from exhaustion or from 
suicide.* 

The treatment of melancholia is very simple. One or 

* The author has not entered upon an enumeration of the morbid anatomy of 
melancholia or mania, as there is nothing but pure hypothesis to proceed upon. The 
vascular theories of Meynert have not been corroborated by others. 



INSANITY. 621 

more competent nurses must be deputed to watch over the 
child and protect it against all harm. It must be fed care- 
fully with a spoon, and will do best on milk, some cereals, 
scraped meat, and eggs. The stomach-tube should not be 
resorted to unless absolutely necessary. Opium in small 
doses, or hyoscyamia in the agitated forms, are the most use- 
ful drugs ; both can be given hypodermically if necessary, 
but they should be discontinued as soon as practicable. Sul- 
fonal, chloralamid, or trional will help to induce sleep and to 
reduce mental excitement. As in cases of mania, a warm, 
full bath is a valuable aid in treatment. 

There is no sufficient reason to remove such children 
from comfortable homes, if isolation at home is possible ; 
but if the environment of the child is an unfavorable one, 
the sooner it is taken to an asylum the better it will be. 

Periodic and Circular Insanity are very exceptional 
occurrences in children. The youngest patient of this class 
the author has seen was a boy, aged eighteen, and all of the 
patients described by Krafft-Ebing, Jacobi, Kelp, and others 
have been near or beyond the age of puberty. Periodic 
insanity consists of successive attacks of mania or of melan- 
cholia, followed by a lucid interval of months or years, and 
then a recurrence of the same conditions. Circular insanity 
is closely allied to this form. As described by Krafft-Ebing 
it is distinguished from the ordinary periodic insanity by 
an alternation of maniacal and melancholy stages followed 
by a lucid interval, and then a recurrence of derangement 
in the same or the reverse order as before. The diagnosis 
of circular insanity can be suspected, but not made, until 
the patient has passed through several cycles. 

The duration of each cycle may vary from several davs 
to several weeks ; but in the author's experience as the dis- 
ease progresses the lucid periods grow shorter and shorter. 
The perodic and circular forms of insanity are of the hered- 
itary degenerative type, and the prognosis in them is far 
less favorable than it would be in ordinary mania or melan- 
cholia. During the maniacal or melancholy state the treat- 
ment would be similar to that described for each con- 
dition. These patients are dangerous to themselves and 
their surroundings, as they pass quickly from one stage to 



622 THE NERVOUS DISEASES OF CHILDREN. 

another. As soon as the circular and periodic character of 
the disease is established, it is best to keep such children 
under the constant observation of an attendant, or else to 
place them in special institutions. 

Cataleptic Insanity. — This term refers to a special 
condition in which a stage of melancholy depression, or of 
maniacal excitement is followed by a peculiar apathetic 
state, during which the patient may assume histrionic atti- 
tudes, or his limbs may remain in any position assigned 
them. Often there is an alternation between melancholy, 
maniacal, cataleptoid, stuporous, and emotional periods ; 
and back of all there is generally an hysterical or epileptic 
disease, and very often the habit of masturbation. A spe- 
cial cataleptoid condition was first described by Kahl- 
baum, and termed " katatonia." It differs from other 
cataleptic states in the greater development of verbigeration 
and the constant aiming at dramatic effect. Spitzka and 
Kiernan, in this country, have recognized the existence of 
this disease, but many alienists deny its claim to be con- 
sidered a special morbid entity. 

The conditions designated as cataleptic insanity and katatonia occur in 
children, as will be illustrated by the following interesting example : 

The patient is a boy, fourteen years of age, who had attained considerable 
notoriety as the promoter of an anti-cigarette league among the public-school 
boys of New York. The mental change came about gradually after a polit- 
ical mass-meeting, at which one of the speakers singled out this boy and 
directed his speech at him. At first he exhibited signs of cerebral exhaustion, 
then developed hysterical convulsions, and finally went into a state of excite- 
ment followed by melancholy depression. In the hospital the boy would at 
first not say a word ; after a few days he began to speak, but only in whispers ; 
he looked frightened, and refused food. While in this stage his limbs, if 
moved passively, would remain in a cataleptic condition. He made very few 
spontaneous movements. He continued in this state for several weeks. In 
addition to the cataleptic state the boy presented distinct hemianassthesia of 
the left side and several anaesthetic areas on the right side. These hysterical 
stigmata remained after the boy had recovered from the stuporous condition. 

At the hospital the boy was kept very quiet, fed carefully, and treated by 
tonic measures. After a lapse of four weeks he began to talk, and to talk 
volubly about his services in the anti-cigarette league and the good he expected 
to effect. His remarks bore all the characteristics of a stump speech as pub- 
lished in the daily papers. When I suggested that an anti-masturbation 
league among boys would do more good than the cause he was promoting, he 
assented very knowingly. 






INSANITY. 623 

As in other cases, so in this one, the cataleptic condition 
is but one of many psychic changes. It is commonly de- 
veloped upon an hysterical or epileptic basis, and often is 
the precursor of paranoia in later years. 

Acute Dementia characterized by a sudden dimi- 
nution of all the mental faculties has been observed in 
young subjects, but hardly before the age of puberty. 
Emminghaus distinguishes an agitated and a stuporous form, 
both representing a primary insanity, which is liable to be 
developed after the acute infectious diseases (typhoid fever, 
scarlatina, etc.), or after severe emotional excitement, early 
cares, exhausting work, or possibly after excessive mastur- 
bation. For a time such children resemble idiots; but after 
several weeks, or months, signs of returning intelligence are 
noticeable and complete recovery ultimately sets in. Such 
patients can generally be treated at home, and do well un- 
der careful feeding and tonics. 

Paranoia.* — Primary insanity is a degenerative psy- 
chosis of the hereditary order characterized by hallucina- 
tions and delusions. The latter are primary symptoms and 
not secondary to the exalted or depressed mood, as in 
mania and melancholia. The delusions or " fixed ideas," be- 
come systematized, and dominate the mental activity of the 
individual to such a degree that they become the mainspring 
of all action. The paranoiac is not amenable to reasoning ; 
and his delusions cannot be dislodged by argument, as 
happens with the temporary delusions of the sane. He 
may have one set of delusions or many. The persistence of 
one or many proves that the entire logical apparatus is out 
of gear. It is wrong to claim that any person is insane on 
one point only ; he may show his insanity in one direction 
chiefly, but his mental derangement is as marked as though 
he had dozens of fixed ideas. 

The systematized delusions of paranoia may be divided 
into two great groups ; first, delusions of persecution ; and 
secondly, delusions of grandeur ; the latter may be sub- 

*The writings of Snell, Westphal, Sander, Krafft-Ebing, and Meynert, have con- 
tributed most to an understanding of this subject. Spitzka treats the subject very 
lucidly under the name " Monomania," preserving an old term, but discarding the doc- 
trine of the " Monomanias" which did so much harm in psychiatry. 



624 THE NERVOUS DISEASES OF CHILDREN. 

divided into the religious, the political, and the erotic type. 
In this form of insanity, with delusions of persecution, the 
patient believes himself to be the victim of circumstances. 
He is made to suffer either for wrongs he has committed 
or for the envy others feel toward him. He has, as a rule, 
been morose and exclusive. He feels that he is being ob- 
served by others ; that every one notices a peculiarity in 
him ; that others can read and control his thoughts ; that 
the newspapers direct their flings at him ; when they speak 
of rascals, of thieves, they mean him. Before long he hears 
the voices of his enemies who are trying to ferret out his 
actions ; he stops up the key-holes and draws the blinds of 
his windows. If his neighbors cannot get rid of him as 
speedily as they wish, they put poison in his food, which 
he refuses to take. He supposes himself the victim of the 
police, of socialists, of a religious sect, who will endeavor 
to influence him by electricity, through the telephone, by 
hypnotizing him, or by forcing him to inhale all sorts of 
noxious vapors. 

Paranoia with delusions of grandeur includes all those 
who imagine themselves destined to fulfil some special mis- 
sion ; the political reformers, the Guiteaus, the religious fa- 
natics, the presidents, the emperors and kings, the Goulds 
and Vanderbilts among the insane, belong to this class 
chiefly. Guiteau, descended from a father who believed 
in Mesmerism and in free-love, was particularly fond of 
reading on religious subjects ; he masturbated at an early 
age and entered the Oneida Community at the age of nine- 
teen ; when twenty-four years old he writes to his father 
saying that he was employed by Jesus Christ & Co. Some 
paranoiacs exhibit signs of the insane neurosis at a still 
earlier day. 

The children who are exclusive, who never care to play 
with others, who pray when their comrades frolic about 
— these are the very ones who develop paranoia later in 
life. Moody, irritable, queer, and " cranky," they go along 
well enough until they have to compete with others in the 
struggle for existence, or until they are overcome by some 
severe grief, by strong emotion, by political or religious 
excitement (the election campaigns, the revival meetings, 



INSANITY. 625 

and the like) ; and then delusions, which may have been la- 
tent for a long time come to the foreground. The paranoiac 
comes of neurotic stock, in which insanity, hysteria, epi- 
lepsy, and chronic alcoholism have been common occur- 
rences. 

Not all children who are morose and exclusive turn into 
paranoiacs, but it is well to look with suspicion and fear 
upon youthful prodigies, who discuss philosophy, or work 
at visionary schemes while other boys are engaged in sport. 
Children, as well as adults, should exhibit a modicum of 
learning and of virtues, and possibly a few vices.* 

Emminghaus describes a form of acute paranoia with 
hallucinations which is said to occur in children after febrile 
attacks, but the cases he quotes are not very convincing ; 
they are to be distinguished from ordinary mania by the 
evidence of hallucinations and by their mode of onset. 

The course of paranoia is eminently chronic ; for a time 
slight remissions may occur, enabling the person to return 
to some regular occupation. He may be able to keep his 
delusions in abeyance for a varying period of time, but they 
will come to the front in the end. The chronic stage is 
established sooner or later, in which he is entirely con- 
trolled by his delusions, and is a fit subject for asylum treat- 
ment. Very little can be attempted in this disease in the 
way of treatment, but if the first traces of the disorder are 
observed in a young boy, a special endeavor should be 
made to provide him with healthful surroundings, to di- 
vert his mind from his own person, and to arouse his 
interest in those things which are the reverse of those he 
is accustomed to brood over. Special teachers, and tours 
to foreign lands may accomplish something ; but disap- 
pointment to parent, teacher, and physician is the most 
common result. 

Moral Insanity. — We need say little of this condition, 
which has given rise to so much discussion. It is generally 
conceded that a lack of the moral sense may be the chief 
feature, but this moral imbecility is generally associated 
with a defect in the intellectual sphere ; hence many of the 

* In this chapter the author has drawn occasionally upon the descriptions given b> 
him in his article on Insanity and Crime, in Hamilton's System of Legal Medicine. 
40 



626 THE NERVOUS DISEASES OF CHILDREN. 

subjects of moral insanity are idiotic or feeble-minded per- 
sons. 

Maudsley, some years ago, went out of his way to defend the rights of 
moral insanity. " It maybe witnessed even in young children, who, long be- 
fore they have known what vice meant, have evinced an entire absence of 
moral feeling, with the active display of all sorts of immoral tendencies, a 
genuine moral imbecility or insanity." But the author is compelled to add 
that " associated with this defect there is frequently more or less intellectual 
deficiency, but not always ; it sometimes happens that there is a remarkably 
acute intellect with no trace of moral feeling." 

There are some, children and adults, whose intellect- 
ual faculties are on a far higher plane than their moral 
qualities, and in whom the latter cannot possibly be fostered. 
In a family, one of five or six children may be the only one 
to resist the influences of religious and secular training. 
Many authors are firmly convinced that the defect in mo- 
rality is to be ascribed primarily to an intellectual defect ; 
opposing views are held by some of the ablest writers, in- 
cluding Lombroso, Maudsley, and Hack-Tuke. Krafft- 
Ebing urges the justice of retaining moral insanity as a 
clinical form, and to this there can be no objections. 

Some years ago I saw a young man at a clinic who had been arrested for 
assault upon his mother, whom he had failed to kill. He was entirely indif- 
ferent to the charge brought against him, and when asked whether he 
thought it was proper to kill one's mother, answered, " You might as well 
kill your mother as anyone else." The young man had received no intellect- 
ual or moral training, no religious instruction, had grown up among the 
most degenerate classes, and had never received the most ordinary moral 
teachings. Naturally the* moral sense was deficient. Such a condition is 
embraced in Mendel's definition of moral insanity as that form which is either 
congenital or acquired in the earlier years of life, and is characterized by im- 
becility associated with a morbid tendency to immoral actions. Binswanger 
holds very correctly that a number of mental diseases lead to " moral idiocy." 

The prognosis of the condition of moral insanity is abso- 
lutely unfavorable ; nothing can be accomplished except by 
placing such children with moral obliquities under the stern 
discipline of a reformatory or an asylum. 

Epileptic Insanity.* — Mental derangement associated 

* Hysterical Insanity has been alluded to in the chapter on Hysteria (pp. 86-88). 
The author was at first inclined to discuss the entire disease in this division on mental 



INSANITY. 627 

with epilepsy has been alluded to in a previous chapter. 
(See page 69.) It is only necessary in this connection to re- 
call the fact that a maniacal attack may take the place of an 
ordinary epileptic seizure, and that such an attack is charac- 
terized by unusual suddenness and violence. Any very sud- 
den development of mania in a child may be regarded as a 
symptom of possible epilepsy, even if other epileptic signs 
are entirely wanting. In addition to these " psychic equiv- 
alents " of an epileptic seizure, epileptic children exhibit a 
marked tendency to idiocy and dementia. I have known 
of the condition of double consciousness in an epileptic 
aged eighteen, but I have not seen it in younger subjects. 
The more pronounced symptoms of epileptic insanity are 
encountered in persons past the age of puberty, but Wilder- 
muth insists that only about twenty per cent, of infantile 
epileptics exhibit anything like a normal mental condition. 

A general psychic degeneration is a most marked feature of epileptic sub- 
jects. The disease is very common among criminals, as are other degenerative 
neuroses. Alcoholism in the parent is a powerful etiological factor. (De- 
jerine states that in 37.7 per cent, of three hundred and fifty epileptics the 
father was a drunkard.) Moreover, the epileptic boy is unable to attend 
school and falls an easy victim to bad associates. 

Paretic Dementia, the serious psychosis of middle life, characterized by a 
progressive dementia, delusions of grandeur, and a long series of physical 
symptoms (inequality and immobility of the pupils, disturbances of speech, 
tremor of the face and hands), is so rare in childhood that we do not feel 
warranted in including it among the forms of insanity occurring in childhood. 
The youngest paretic I have seen was in a man, aged nineteen. Spitzka 
observed one at eighteen years, Trumbull observed the disease in a boy of 
twelve years, who had an attack of hemiplegia at the age of ten years. The 
autopsy revealed the changes of paralytic dementia. The cases thus far ob- 
served in youthful subjects did not take as rapid a course as in the adult type. 

The author would make no mention of the disease, were it not for an ex- 
perience he had some years since with a young man who in the course of a 
few weeks became exceedingly hilarious, developed the delusion that he was 
enormously wealthy, showed distinct tremor of speech and of hands, inequal- 
ity of the pupils, and became extravagant and grandiloquent. The diagnosis 
of paralytic dementia was made by competent neurologists ; the boy was sent 
to an asylum, where all his symptoms disappeared after a few weeks. He 
has been entirely well since. 

diseases, but hesitated to do so because the marked psychic forms of hysteria are nor 
often observed in children. The mental changes in chorea have been referred to on 
page 116. 



628 THE NERVOUS DISEASES OF CHILDREN. 

Masturbation and Insanity. — There is not sufficient 
reason to erect a special type to be called " Masturbatic In- 
sanity," for the habit is present in many different forms of 
insanity, particularly in young subjects. But the presence 
of this etiological or complicating condition is always no- 
ticeable, and leaves a distinct impression upon the develop- 
ment of the various psychoses. In many cases of acute 
dementia in the young, in hebephrenia or the insanity of 
pubescence, it is often the controlling factor. The author 
has had under his observation young masturbators who at 
the ages of ten and twelve years displayed the first effects 
of masturbation, and at the ages of sixteen to twenty years 
presented the typical symptoms of hebephrenia (mental 
enfeeblement, silliness, depression, general restlessness, and 
irritability), and gradually developed marked idiocy. There 
is such a striking resemblance between various forms of in- 
sanity due to masturbation that one is sorely tempted to 
constitute them a special group. The following account 
is typical of many the author could give : At the age of 
twelve or fourteen years, if not earlier, the boy either in- 
stinctively or through the force of bad example begins the 
habit. For a time it has no marked effect upon him ; he 
continues in his studies, but does not get on quite as well 
as he formerly did. For this he finds all sorts of excuses. 
He is supposed to be, and often is, subject to severe head- 
aches ; is tired in the morning, late in rising, loses his 
pleasure in out-of-door sports. If detected at this stage 
and convinced of the viciousness of his habit he may make 
a determined effort to stop ; sometimes he succeeds; more 
often he fails. From this period on, lack of concentration 
upon any mental effort, general irritability, disturbed sleep, 
loss of flesh, are the prominent symptoms. Morbid con- 
ceptions are often troublesome, and at times also lead to 
delusions of persecution. A young man under my care 
who would gaze for hours upon a clock heard it say, " De- 
tec-tive, De-tec-tive," and imagined himself run down by 
them, but was even then too stupid to give any reason for 
such persecution. The patient leads an aimless, silly exist- 
ence ; will pore for hours over books without reading them ; 
does nothing of his own initiative, but if forced to walk 






INSANITY. 629 

or to take exercise of some sort does everything in a me- 
chanical fashion. If he be the son of wealthy parents, the 
effort is made to distract him by travel, but all is in vain ; 
he takes no interest in anything and simply watches for 
the opportunity to indulge in masturbation. Complete 
idiocy and dementia are the ultimate result. Reference 
has been made to boys, but girls also fall victims to the 
habit, and the symptoms they present are similar to those 
specified before. The prognosis of all forms of insanity due 
to masturbation is extremely unfavorable. I have succeeded 
in checking the habit in relatively few cases. Many patients 
continue on their evil course in spite of all precautions. It 
is, if anything, more difficult to control the habit in girls 
than in boys. If the person's reason and sense of shame 
can be appealed to the chances of curing him of the prac- 
tice are best; the previous mental calibre is an important 
factor ; the successful cases I have seen have been in col- 
lege boys and other bright lads. If the habit has been ac- 
quired by imitation it can be checked more readily than if 
it is the result of an innate instinct, and the sign of a degen- 
erated mental state. Extreme watchfulness is the only 
possible means of effecting a cure. 

The prognosis of insanity in children has been discussed 
with reference to each type mentioned. It is well to em- 
phasize the fact once more that mental derangement in 
childhood is recovered from more frequently than is the 
case with the insanity of later years. But a child that has 
once been insane is a tender plant that needs special care ; 
and unless intelligently guided is liable to break down 
under any great emotional strain or unusual excitement. 

A word in addition to what has been said on the treat- 
ment of the various types of insanity. The doctrines of 
heredity and of degeneration are taking a firm hold of the 
medical mind. Psychologically they may be true enough, 
but no one has yet proved that morbid hereditary influ- 
ences cannot be overcome or counteracted. If a child has 
an unfortunate hereditary predisposition to disease, or even 
to crime, it is the physician's first duty to give it, it pos- 
sible, every advantage which children without any he- 



63O THE NERVOUS DISEASES OF CHILDREN. 

reditary taint enjoy. The separation of children from hys- 
terical, epileptic, or otherwise degenerate parents, is not 
insisted on often enough. 



BIBLIOGRAPHY. 

(The text-books of Spitzka, Hammond, Krafft-Ebing, Schiile, Kirchhoff 
(English translation) ; the monograph of Emminghaus, Die psychischen 
Storungen im Kindesalter, 1887, which is the most complete work on the 
subject ; the articles on Insanity in Children, by Spitzka, in Keating's Cyclo- 
paedia, and by Mills in L. Starr's Text-Book of Diseases of Children.) 

Alexander, Harriet C. B. : Alienist and Neurologist, 1893, 1894. 

Binswanger : Volkmann's Samml, No. 299. 

Bucknell and Tuke : Psycholog. Med. London, 1858. 

Cohn : Arch. f. Kinderhk., vol. iv. 

Down : Mental Affections of Childhood and Youth. London, 1887. 

Esquirol : Maladies Mentales, vol. L, 1838. 

Hayem : Statistical Inquiries, etc. Erlangen, 1876. 

Hurd : Boston Medical and Surgical Journal, cxxxi., 1894. 

Kahlbaum : Katatonia. 1874. 

Maudsley : International Scientific Series. Fourth Ed., 1881. 

Mendel: Die Manie. Leipzig, 1881 ; and articles in Eulenburg's Real-En- 
cyclopsedie. 

Meyer, L. : Arch. f. Psych., vol. i. 

Meynert: Centralbl. f. Psychiatrie, vol. iii., 1881. 

Mills, C. K. : Transactions of the Medical Society of Pennsylvania. Phila- 
delphia, 1893. 

Morel : Maladies Mentales. 1853. 

Moses : Dissertation. Strassburg, 1892. 

Prichard: Treatise on Insanity, 1835 (with especial reference to Moral In- 
sanity). 

Sachs : in Hamilton's System of Legal Medicine. 

Sander : Arch. f. Psych., vol. L, p. 389. 

Scherpf : Jahrb. f. Kinderheilkunde, N. F., vol. xvi. 

Sinkler : University Medical Magazine, 1892-93, vol. v. 

Steiner : Compendium, etc. Leipzig, 1872. 

Taylor: Arch, of Pediatrics, No. 11, 1894. 

Turnbull : Journal of Mental Science, Oct., 1881. 

Westphal : Gesammelte Abhandlungen, vol. L, p. 446, and numerous articles 
in the Arch. f. Psychiatrie. 

Wilmarth : Journal of American Medical Association. Chicago, 1894. 



CHAPTER XXXIII. 

IDIOCY AND IMBECILITY. 

By idiocy we designate a permanent and complete im- 
pairment of all the intellectual faculties. Imbecility and 
feeble - mindedness denote lesser degrees of mental defi- 
ciency. Both these terms are used to designate mental de- 
ficiency in a brain not yet fully developed, whereas the 
term " dementia " is applied to those states in which all 
the faculties are lost after they have been normally de- 
veloped. Such dementia may occur at times in relatively 
young persons. 

Idiocy is of special interest, not only because of its frequent occurrence, 
but also because it demonstrates an extreme psychic condition due, in many 
instances, to tangible changes in the brain. In this respect it is not unlike 
general paresis, and the mental conditions included under these two terms 
are practically the only two psychic diseases whose morbid substratum is 
tolerably well known at the present time. In one, the highest structures 
of the brain have become disorganized and are undergoing dissolution after 
having attained normal development, while in the other defective growth or 
involution takes place before a full development has been reached. 

In former years idiots were much neglected ; they were 
considered an unfortunate class for whom but little could 
be done, and whose actual condition was scarcely worth 
studying ; but during the past twenty-five years or more 
an entire change has taken place and it is fair to say that 
no class of patients has been more frequently the subject 
of study than those with defective cerebral development. 

The earliest impetus was given in this direction by the French alienists. 
chief among them being Esquirol ; a little latter the elder Seguin called at- 
tention to the possibilities of improvement in these patients and to the proper 
methods of training; and within the past decade or two, the interest in the 
morbid conditions underlying idiocy has led to numerous able contributions. 



632 THE NERVOUS DISEASES OF CHILDREN. 

Among English authors the names of Crichton Browne, West Maudsley, and 
Langdon Down, of Clouston, Ireland, and Shuttleworth, deserve special men- 
tion ; in France, Bourneville has been the most successful student of idiocy ; 
in Germany, the works of Schiile, of Krafft-Ebing, of Emminghaus, and 
above all, of Griesinger, have contributed most to the advance of our special 
knowledge of this subject, and in this country idiocy has been studied care- 
fully by Ray, Seguin, Mills, Kerlin, Spitzka, Hurd, Brush, and the author. 

Classification. — The discussion of idiocy has been not 
unlike that of insanity. Each author has felt called upon 
to subdivide the subject and to establish a rational classi- 
fication. The result has been that no two authors have en- 
tirely agreed. One has attempted to give a classification 
based upon the etiology of the condition, another upon the 
pathology. Down has attempted a division based upon 
ethnic standards, the Mongolian, the Malay, the Indian, and 
the Ethiopian types. 

For purposes of convenience we will divide the subject 
as follows : 

T tt j-. tj- i Ca) Congenital. 

I. Hereditary Idiocy } v ' 5 , 

j (b) Developmental. 

( After traumatic injuries (including birth idiocies). 

II. Acquired Idiocy.. \ After convulsions. 

( After infectious diseases. 

III. Myxoedematous Idiocy. 

I. (a). Hereditary Congenital Idiocy includes those condi- 
tions in which the brain at birth is deficient, although 
this defect need not be apparent for some weeks or even 
months after birth. In the chapter upon Arrested Cerebral 
Development I have enumerated some of the anatomical 
conditions with which idiocy is associated, and to this part 
of the subject we shall recur later on. These congenital de- 
ficiencies may be the result of disease during the intra- 
uterine period. 

Congenital idiocy is a common occurrence in families 
with marked neurotic taint. Parents who suffer from some 
form of insanity, from hysteria, epilepsy, or chorea are apt 
to engender idiots. Among other predisposing conditions 
alcoholism and syphilitic infection of either parent are by 
far the most frequent. The importance of alcoholism in 



IDIOCY AND IMBECILITY. 633 

particular cannot be overrated. The alcoholic habits of 
the father at the time of procreation are surely a potent fac- 
tor. Syphilis of the parent must be taken into account. 
Premature delivery is not uncommon in syphilitic cases, 
and an imperfectly developed brain at the time of birth 
may be the cause of idiocy. 

Intermarriage is supposed by many to be a direct 
cause of idiocy in children, and by others the influence of 
such marriages is absolutely denied. The truth lies mid- 
way. In families without a hereditary taint intermarriages 
may be quite harmless, but if there is any, and even the 
slightest taint of insanity, or of any other serious nervous 
affection, that taint becomes intensified by such an union ; 
and since few families are entirely free from every such 
tendency the rule holds good that in the majority of cases 
intermarriages are harmful. 

Traumatism during pregnancy is another factor in the 
development of idiocy in the child. As the influences 
brought to bear are prenatal in origin, these cases may be 
included under the heading of hereditary idiocy ; and there 
is all the more reason to do this as such traumatic injuries 
are more likely to be harmful to the normal development 
of a child's brain in families with neurotic taint than in 
families whose history is entirely negative. 

I. (b) Developmental Idiocy includes that class of cases in 
which the idiocy becomes apparent at certain well-marked 
periods of life — during dentition, for instance, and at pu- 
berty. 

II. Acquired Idiocy. — This class includes a very large 
number of conditions, due to the most varying accidents 
and diseases. First and foremost, although the cause is 
operative at the time of birth, we must include that large 
number of idiocies due to traumatism during labor. The 
same conditions which give rise to cerebral birth palsies 
(prolonged labor, instrumental delivery) also give rise to 
birth idiocy. Other things being equal the application of 
instruments is not nearly as harmful as excessively pro- 
longed labor. In the histories of children who have be- 
come idiotic, it is often stated that the child was asphyxi- 
ated at birth ; that it had frequent spasms during the earlier 



634 THE NERVOUS DISEASES OF CHILDREN. 

weeks of life, and that it exhibited marked rigidities and 
palsies of the extremities. 

According to Mitchell, in 57 of 494 cases of idiocy labor lasted for more 
than thirty-six hours ; 4 of them were born with unusual haste ; in 22 pases 
forceps were applied, and 9 of these showed the impressions of the for- 
ceps on the head after birth ; 4 of them were born by version ; in 6 cases 
there was breech presentation ; 1 1 were twins, and 9 were born prematurely ; 
29 were born asphyxiated and supposed to be dead ; 89 were the last children 
of their respective mothers. 

Birth idiocy is allied to another form due to injuries to 
the head later in life ; a fall from a bench, from a chair, 
from a cradle, is often sufficient to cause serious disturbance, 
more particularly in those children who are predisposed 
by inheritance to mental disease. It is possible that the 
easier rupture of the blood-vessels in those who have in- 
herited a syphilitic or alcoholic taint may account for the 
fact that while some children escape injury after severe 
falls others are seriously affected by relatively slight acci- 
dents. 

Among the causes of acquired idiocy none is more im- 
portant to my mind than convulsions. The entire devel- 
opment of a child may be normal until a convulsion occurs, 
whether as a result of some intestinal derangement or as 
the precursor of an acute infectious disease. From this 
time onward mental decadence sets in, and a child that 
was previously healthy and of normal mental development 
begins to exhibit more and more marked mental defects 
until it reaches the condition of complete idiocy, in which it 
may remain for many years. There is no difficulty in ex- 
plaining this occurrence, for the tremendous venous stasis 
that occurs during the acme of a convulsion is sufficient to 
cause rupture of the pial blood-vessels, and a very consider- 
able hemorrhage may follow ; meningo-encephalitis, termi- 
nating in a general sclerosis, is the natural result and the 
direct cause of the mental defect. The acute infectious 
diseases are often responsible for the development of idiocy. 
In what manner this cerebral change is brought about is 
not easy to state, but I believe that the cases thus produced 
are relatively few, and that of these some are due to an in- 



IDIOCY AND IMBECILITY. 635 

fectious encephalitis, and others to the convulsions accom- 
panying these acute infectious diseases. 

Idiocy is developed not infrequently after acute menin- 
gitis early in childhood. Idiocy, blindness, deaf-mutism — 
all these conditions separately, and sometimes conjointly, 
are the unfortunate results of early meningeal disease. The 
explanation of this condition is relatively simple, for the 
meningitis and the meningo-encephalitis, if they last long 




Fig. 160. — Hydrocephalic Idiot. (Peterson.) 

enough, may lead to a general sclerosis and atrophy of the 
cortical substance, which will prevent the further normal 
development of the brain. 

The association of idiocy with hydrocephalus is com- 
mon, but as this condition is generally a secondary state, 
both the idiocy and the hydrocephalus are the result of the 
primary disease. (See Fig. 160.) With the gradual increase 
of the hydrocephalic fluid, however, the cortical t unction 
naturally becomes impaired ; there are therefore lew chil- 
dren with unusually large heads whose mental condition is 
at all normal, though it is quite remarkable to what extent 
the hydrocephalus may increase before an absolute aboli- 



636 THE NERVOUS DISEASES OF CHILDREN. 

tion of function takes place. This is true not only of the 
general mental condition, but also of the special sensory 
functions of the cortex. 

Idiocy and epilepsy are intimately associated with one 
another. In all cases in which epilepsy has developed early 
in life, or in which the epileptic seizures are frequent, there 
is a natural tendency to mental deterioration. In the adult 
we speak of epileptic dementia, in younger persons of epi- 
leptic idiocy. There are no doubt gross changes in the 
brains of such subjects, and in many the seeds of both the 
epilepsy and the idiocy may be traced to injury either at 
birth or during the first years of life. Thus not only the 
idiocy but the epilepsy, as well as the palsy, may be the re- 
sult of meningeal hemorrhage occurring during labor or in 
very early childhood. 

Symptoms. — The chief characteristic of idiocy is the 
lack of ordinary mental conceptions. The brain is not able 
to receive impressions from the outer world, and if such 
are received, it is not able to utilize them in anything 
like normal fashion, nor to form concepts or judgments. 
In some instances the brain represents an entire blank, in 
others a few impressions have been received, and these 
have been developed into imperfect concepts. This abso- 
lute mental deficiency is present in typical idiots, but there 
are varying degrees of mental deficiency or of mental devel- 
opment in imbeciles or feeble-minded persons. 

It is scarcely necessary to cite cases in order to establish 
the clinical features of complete idiocy, for they are too well 
known even to laymen to need elucidation. 

If idiocy is complete the child or the adult may not be 
able to recognize its own parent. He fails to recognize any 
object or the use of such, and of course is not able to under- 
stand or appreciate what is said to him. In extreme in- 
stances the idiot is practically nothing more than a highly 
organized vegetating organism, truly animal-like in all his 
actions without the slightest trace of human intellect. 
Through faulty development of speech the defect in the 
general mental condition of the child first becomes notice- 
able. If there is a lesser degree of idiocy, the child may 
master a few concepts, may understand a few uttered 



IDIOCY AMD IMBECILITY. 637 

sounds, or may be able to pronounce such simple words 
as mamma, papa, and the like. He may recognize the 
use of objects, may be capable of slight training and in- 
struction, so that the ideas of cleanliness, and of mine and 
thine may be impressed upon his mind. 

From these rather marked forms of idiocy and mental 
imbecility there is every possible gradation until we reach 
those types in which speech is only a little deficient, but 
the mental horizon is evidently narrowed down so that rel- 
atively few concepts are formed, abstract ideas are almost 
entirely wanting, and the imperfect mental status is deter- 
mined by the lack of ordinary motives for action, by the 
awkwardness in intercourse with other people, and above 
all by the inability of the individual to cope with others in 
the struggle for existence. The inferiority of the individ- 
ual is discovered not in the family circle in which all con- 
ditions are favorable, but when the child is thrown into 
competition with others at school. If the boy or young 
man is compelled to seek a living for himself, the feeble- 
minded youth is pushed to the wall under such condi- 
tions. That such persons, moreover, cannot appreciate the 
reason for their failure to make a success of life'goes with- 
out saying. That they are rarely capable of developing 
the higher religious and moral ideas is equally true. The 
criminal classes are recruited largely from the category of 
feeble-minded persons in whom the ordinary ideas of right 
and wrong have not been engendered by early training. 

The general deficiency in intellect is supposed to be offset occasionally by 
a peculiar development of the mind in some one direction. Stories to this 
effect have been common, and have been repeated by one author after the 
other ; Griesinger's case has become rather famous of an idiot who was said 
to have constructed a perfect model of a man-of-war without having had 
any conception of geometrical designs. Many idiots are said to possess spe- 
cial talent in the use of cards. Drobisch described an idiotic boy, who after 
reading over a single page was able to repeat word for word, even if it was 
in Latin, which he did not at all understand. I have had under observation a 
young imbecile, a boy aged fourteen, who takes special delight in reading and 
remembering each sign that he passes on the streets, and he can without 
difficulty repeat fully two hundred names in the order in which they occur 
on the avenues. He has a true passion for signs, and as he passes along the 
streets his attention is riveted upon nothing else. Some of the most astound- 



638 THE NERVOUS DISEASES OE CHILDREN. 

ing lightning calculators have been weak-minded, if not imbecile, in everything 
else excepting memory of figures. It is not fair, however, to turn this about 
and to claim that every lightning calculator must necessarily be an imbecile, 
for there is no doubt that some normal minds can, by special training, reach 
an inordinate development in some one particular direction. 

As we are interested chiefly in the mental condition of* 
children, we must devote a little more time to the early rec- 
ognition of idiocy. In cases of very marked mental defi- 
ciency the disordered condition of mind can be discovered 
in the first months of life. In fact if parents and physicians 
had a proper understanding of such conditions, the mental 
defect could be discovered very much earlier than it gen- 
erally is. It is most instructive in this connection to con- 
sider the observations of Preyer, who, in his famous treatise 
on the psychic development of the child, shows how early 
the mental processes of a healthy child can be discerned. 
It is a surprise to most of us to learn from him that a child 
in the second day of life was able to distinguish between 
light and darkness ; that at a very early day it appreciates 
the sounds of words, and that in its cooing utterances it 
exhibits evidence of considerable cerebration. Very few 
people wiU, of course, observe children as Preyer observed 
his own child, but mental deficiency can, as a rule, be made 
out if the parent or the physician take the trouble to com- 
pare the actions of a child with the average healthy child 
at the same age. It is important, therefore, to note the time 
of life at which children may be expected to exhibit certain 
signs of intelligence. (See page 7.) If children have not 
acquired speech at the end of two and a half years, and par- 
ticularly if they fail to understand what is spoken, it is fair 
to infer that there will be a distinct mental defect later on. 

Wildermuth has taken the trouble to tabulate a number of the more im- 
portant stigmata of degeneration commonly found in idiots. Eighty-two per 
cent, of all idiots according to these statistics present some such signs. There 
were abnormal conditions of the fundus in 6 of 142 cases ; malformations of 
the external ear in 53 ; abnormal position of the teeth in 32 ; flattening of 
the hard palate in 1 1 ; highly arched palate in 30 ; prognathism in 9 ; ex- 
cessive thickening of the skin in 9 ; asymmetry of the face in 25 ; and abnor- 
malities of the genital organs in 8 cases. Among the functional stigmata 
Wildermuth refers to anomalies of the tendon reflexes in sixty per cent. ; 
inco-ordination of the lower extremities in ten per cent. ; and in six per cent. 



IDIOCY AND IMBECILITY. 



639 






squinting occurred. It is a curious fact that these signs of degeneration are 
present not only in the truly congenital forms of idiocy, but also in those 
which have been acquired some time after birth. 

The mere appearance of the child or of the youth will 
often be sufficient to lead to the suspicion of idiocy without 
an examination of the 
mental condition itself. 
The entire absence of 
speech, or of defective 
speech in many cases, will 
point the same way. Cau- 
tion should, however, be 
exercised in not mistaking 
deaf -mutism for idiocy, 
and if a child cannot 
speak, careful examina- 
tion should be made in 
other ways to determine 
its intelligence ; not rare- 
ly, however, deaf-mutism 
and idiocy are combined, 
and at times the former 
has been the direct or in- 
direct cause of the latter. 

Among idiots the de- 
fects of skull formation 
are of especial interest. 
The microcephalic skull 
is particularly frequent, 
and is either small in all 
its dimensions or ample 
in some and very defi- 
cient in others. (See Fig. 
161.) The transverse and 

occipital diameters may be entirely normal, indeed the 
horizontal circumference may be up to the average, and 
yet if some such heads are examined it will be found that 
the frontal portion is unusually small, possibly receding, 
while the middle and occipital portions may be entirely 
normal. Such discrepancies and such asymmetry should 




Fig. 161. — Congenital Idiot ; Microcephalic 
Skull ; Extreme Contractures of Adductor 
Muscles of Thighs. (From a photograph 
kindly furnished by Dr. Peterson.) 



640 THE NERVOUS DISEASES OF CHILDREN. 

be carefully noted, for it is more important than slight 
deficiencies in the total measurements. Smallness of the 
anterior half of the skull with receding forehead proves 
either that there is very little room for the brain, or that 
the brain being small requires very little space. Asymmetry 
of the skull is particularly frequent, as has been shown by 
Fisher and Peterson, in those cases in which idiocy is asso- 
ciated with early infantile hemiplegia. Another point to be 
remembered is that irregularities in the structure of the 
skull are not uncommon in healthy individuals, and that as 
long as the cubic contents of the^ cavity of the skull are 
near the average, defects of one kind or the other appear to 
play a very small part. In various parts of this book I 
have referred to my belief that the growth of the skull is 
dependent largely upon the condition of the brain within, 
and this accounts for the fact that in idiocy acquired after 
convulsions the growth of the skull ceases with the dis- 
turbed condition of the brain; microcephalus is found 
among acquired idiots as well as among congenital idiots. 

The general restlessness of idiots is characteristic. In 
my lecture-room I allow the idiot children full sway in 
order to demonstrate this special feature to my class. They 
are about the only patients who concern themselves little 
about the presence of the students, and who roam about the 
entire room in an uneasy fashion, taking hold of everything, 
pulling down what they can, and always fearless of the 
consequences. If they cannot employ themselves in any 
other way, they will keep up a constant motion with the 
fingers, twisting and braiding them, allowing the nails to 
scrape the skin off the fingers. They bite the nails and are 
apt to tear their garments. There is frequently dribbling 
and drooling, and if opportunity is offered these children are 
particularly fond of putting their tongues to the window- 
panes, or any other cold object. The general awkward- 
ness of the movements, excitability and irritability of tem- 
per, together with a peculiarity of carriage and a blankness 
of facial expression complete the clinical picture of idiocy 
and imbecility. 

Imbeciles of lesser degree often escape detection ; some of them are suffi- 
ciently conscious of their defects to conceal them in the presence of others. 



IDIOCY AND IMBECILITY. 64 1 

As they grow older the deficiency in the mental and moral make-up is evi- 
denced in the excessive development of the animal appetites. Masturbation 
is developed in early years, the effect of which adds to the mental deteriora- 
tion. If the sexual appetite is thoroughly aroused, gratification is sought in 
the most outrageous fashion, and intercourse may be attempted with children 
or old women. Giraud referred to an idiot who attempted to rape his own 
sister. All the ordinary feelings of shame and modesty are wanting. In some 
instances the defect is a little more marked in the emotional or moral spheres 
than in the intellectual. Such a condition has generally been termed one of 
moral imbecility ; but it is always associated with a decided intellectual defect. 
Weak-minded and imbecile children commit crimes either from defective 
judgment as to the consequences or from the simple desire to gratify their 
passions and impulses. The desire to witness a grand spectacle or to 
revenge himself upon others, has led many an imbecile to set fire to houses, 
regardless of all consequences. 

Pathology. — We have stated that idiocy is rarely a 
primary condition, that it is frequently associated with 
other conditions which point to serious brain trouble. A 
complete account of the pathology of idiocy would include 
the terminal stage of very many different brain diseases. 
While it is interesting to note these various terminal states 
it helps us but little in determining the primary lesion. 
Thus'Wilmarth has described one hundred brains of idiots. 
Among these were found conditions of sclerosis with atro- 
phy, of tuberous sclerosis, of general diffuse sclerosis. He 
described, furthermore, brains in which the most marked 
features were degenerative changes in the vessels and higher 
nerve-cells. He also mentioned hydrocephalus, general atro- 
phy, and the like, so that it would be difficult from this 
enumeration to make any sort of inference as to the primary 
morbid state. The attempt should be made to distinguish 
between the primary and terminal states, and thus help us 
to push on, however little, toward a final solution of the 
question. I believe we shall do best if we accept the clini- 
cal division of idiocy adopted in this chapter, and endeavor 
to arrange the known morbid processes as far as possible 
under these larger clinical subdivisions. 
41 



6_p 



THE NERVOUS DISEASES OF CHILD REX. 



Pri—arv Lts::r.s. 






Hereditary idiocv 



Acquired idiocy, 
(a) Birth palsies. 



(b) After acute dis- 
eases and convul- 
se ns. 

(c) From tr aum a- 
tism. 



Large defects ; hemicephalus 
or entire absence of a con- 
siderable portion of a hemi- 
sphere. 

z:--r.x. :iefe;:s *: :rer.:erh- 
aly) ; most frequently in 
motor regions. Single or 
double. 

Small brain (microcephalus) ; 
all parts equally developed, 
or arrest of development 
more marked in frontal and 
occipital portions than in 
other parts. 

Agenesis corticalis ; brain ap- 
parently normal or showing 
only slight changes in ex- 
ternal configuration ; mal- 
development and disintegra- 
tion of cells and fibres of 
cortex. 

Intra-uterine, inflammatory, or 
vascular disturbances, such 
as encephalitis, thrombosis, 
an :. ':.-::. i~::.:z-. 

Meningeal hemorrhage. 



Meningitis. er.cerh.V.::;; sim- 
ple or hemorrhagic) ; throm- 
bosis (specific endarteritis). 

Meningeal hemorrhage ; en- 
cephalitis. 



Same ; often compensator)- de- 
velopment of hydrocephalus 
with large cysts. 

Same ; with addition of hydro- 
cephalus, local cysts, and' gen- 
eral sclerosis with atrophy. 

Small brain, often hard and 
sclerotic, sometimes compen- 
satory hydrocephalus. 



Same ; no sign of infiamma- 
tory conditions, blood-vessels 

scanty; some external hydro- 
cephalus. 



Meningo - encephalitis ; sclero- 
sis, localized or diffuse : gen- 
eral atrophy ; cysts. 



Meningo - encephalitis, local 
cysts, sclerosis, and general 
atrophy. 

Meningo - encephalitis, sclero- 

s:s. diffuse atrophy. 

Meningoencephalitis, z'z s ; rss. 
sclerosis, and atrophy. 



That there are other conditions which occasionally lead 
to idiocy cannot be doubted, but thev are. I am sure, not 
nearly as important nor as frequent as those mentioned in 
the above table. If hereditary syphilis is the etiological 
factor it is more than Likely to give rise to idiocy through 
the medium of local meningitis, encephalitis, or of throm- 
bosis With its subsequent changes. Mills insists on idiocy 
of toxic origin, and includes under this term cases due to 
acute poisoning, or those following acute infectious dis- 
eases, such as measles, scarlet fever, and the like. 

Diagnosis. — Idiocy is recognized easily enough, except 
in very young children ; a comparison with the attainments 
of other children of the same age will help to establish the 
fact of an inferior mental development. If a child presents. 
in addition to the mental symptoms, any of the physical 
stigmata of degeneration, the probability of its remaining 






IDIOCY AND IMBECILITY. 643 

an idiot is very great. Defective development of speech 
after a child has passed the third year is also an important 
aid in diagnosis, if this condition is not the result of deaf- 
mutism. 

The Prognosis in the majority of cases of idiocy is bad. 
If a child has not learned to speak, and has not acquired 
the simplest concepts at the age of three or four years it will 
remain backward for all time. In the case of imbeciles of 
lesser degree ,the prospect is not quite so gloomy. In this 
condition more can be expected from the careful training 
on the part of parents and teachers. 

Treatment. — The treatment of idiots is, as a rule, re- 
stricted to watching over their physical development and 
securing for them proper hygienic surroundings. In fam- 
ilies such children are a torment to their parents and a bad 
example to brothers and sisters. Their separation from 
home is advisable, and should be urged in spite of all senti- 
ment to the contrary. The patients enjoy the greater 
liberty which special institutions afford them, and the family 
is better off without them. 

Imbeciles and weak-minded children require more care- 
ful consideration. By the most diligent training at the 
hands of experienced persons much can be done for them 
to bring their behavior and their physical condition as 
closely as possible to that of the average child. I am a 
thorough believer in pedagogic methods and of such only. 
If at all possible, such children should be intrusted at a very 
early day to a competent teacher, who will take the time 
to study the peculiarities of the individual, and who will 
fasten upon and endeavor to develop the few signs of an 
intellectual awakening. It is often surprising to see how 
much can be accomplished with these unfortunates. In 
some instances, however, the task is a hopeless one, and all 
the evidences of imbecility can rarely be eradicated. By 
careful calisthenic exercises, and proper gymnastic training, 
much can be done to avoid the outward appearance of im- 
becility to which parents so seriously object. It special at- 
tention is paid to the development of speech, defects in this 
direction can be corrected. The larger institutions, such as 
the Bicetre in Paris, and the various institutions for the 



644 THE NERVOUS DISEASES OF CHILDREN. 

feeble-minded in this country, attain a fair measure of suc- 
cess. But, after all, only those who possess a modicum of 
intellectual development can be benefited by these meth- 
ods, whereas the severer grades of imbeciles and of idiots 
cannot be improved by them. 

Cranial surgery has been looked to as a final resort. If 
the imbecility or the idiocy is distinctly due to a small skull, 
and this small skull is the result of a premature synostosis 
of the sutures, the surgical procedures, as practised by Lan- 
nelongue, Keen, Gerster, and others, may be allowable ; but, 
after all that can be said on this subject has been heard, 
there is but little reason to expect much improvement. 
The slight changes that have been noticed and have been 
reported in children after craniectomy scarcely warrant 
the procedure, and, as has been intimated in a previous sec- 
tion, the dangers of the operation are so great that crani- 
ectomy should only be practised at the urgent request of 
the parents, and after every other method has been given a 
fair trial and has failed. In those patients in whom there is 
evidence of a defective cerebral development, independently 
of the condition of the skull, any operation is, on a priori 
grounds, entirely useless. 

Medication by drugs is rarely beneficial ; yet some phy- 
sicians will undoubtedly be impelled by the evidence of an 
existing anaemia, of rickets, or of hereditary syphilis, to ex- 
hibit the drugs commonly used in these conditions. 

III. MYXEDEMATOUS IDIOCY — SPORADIC CRETINISM. 

This condition, which was well described by Bourneville a few years ago, 
deserves special mention. The mental condition is a very striking feature of 
these children, but it is simply a part of the general stunting of the physical 
and mental growth. The condition itself, although a very rare one, is of 
more interest at the present day in connection with the satisfactory studies 
that have been made regarding the cause and the treatment of myxcedema. I 
have associated myxcedematous idiocy with sporadic cretinism, because the 
two seemed to me to be very closely related, the cretin representing, how- 
ever, a more intense development of the affection than is met with in those 
cases described as myxcedematous idiocy by Bourneville. Cases of sporadic 
cretinism in every way resembling the endemic cretinoid cases, so common 
in the mountainous districts of Europe, occur also in a few American re- 
gions, as in the mountains of Vermont and California. The cases which we 



IDIOCY AND IMBECILITY. 



645 



have the opportunity to see in the larger American cities are from the for- 
eign element of the population, and though we speak of them as cases of 
sporadic cretinism, they occur generally in descendants of families who have 
lived in regions in which cretinism has been endemic. 

The child, whose picture is reproduced in this chapter, is a typical case of 
this kind. The boy was born of healthy Prussian parents, who immigrated 
to this country some seven or eight years ago. He has always been healthy, 
but ever since he was a year and 
a half of age, the parents have 
been struck by the fact that 
he did not seem to continue 
growing as the other children 
in the family did. This stunt- 
ed physical growth was asso- 
ciated with an impairment in 
the development of speech and 
of all other faculties. 

The child might at the pres- 
ent day, at the age of twelve, be 
taken to be three, or not much 
more than three years old. The 
boy has learned to speak a few 
words, is able to call his par- 
ents and some near relatives. 
His affections are well devel- 
oped, as was evidenced when 
he was separated from his father 
and placed in the hospital, and 
he shows considerable aptitude 
in playing with other children, 
but not quite as much as a 
healthy child of three years of 
age would. As for his mental 
condition, it is not equal to that 
of a child of three years ; be- 
yond the expression of his sim- 
ple wants, he exhibits remarka- 
bly few signs of mentality. 

The disease is characterized 
by a peculiar glossy appearance of the skin, prominent lips, receding fore- 
head, and a peculiar, stubbed nose. Compared with the rest of the body the 
stomach is inordinately large ; the tongue is relatively thick, and very often 
protrudes from the mouth. All children with myxedematous disease so 
closely resemble each other that they might be supposed to belong to one and 
the same family. The examination of the throat fails to reveal any trace of 
the thyroid gland. 

Etiology. — The etiology of myxedematous idiocy, as well as of spora- 




FlG. 162. — Caseof Myxoedema with Idiocy. Pa- 
tient Twelve Years Old ; Dwarfish in Stature. 



646 THE NERVOUS DISEASES OF CHILDREN. 

die cretinism, is practically unknown, except that it is 1 very apt to occur in 
mountainous regions, and particularly in regions in which the water contains 
a great deal of salts of lime and magnesia. But this is evidently not the 
only explanation, for the children of persons who have emigrated from such 
districts have often been affected with the disease although subject to en- 
tirely different atmospheric and tellurian conditions. The frequent intermar- 
riages between people in isolated mountainous districts also has its bearing 
upon the hereditary transmission of these diseases.* 

Diagnosis.— Myxedematous idiocy cannot very well be mistaken for 
any other condition. It is only in the earlier years of life, when the stunted 
growth and the peculiar expression of the child are not yet fully marked, that 
the true cause of the idiocy may not be suspected. But the arrest in the 
general growth and the peculiar appearance of the skin will give the clew to 
the true state of affairs. 

Prognosis.— The prognosis of this condition was extremely grave until 
very recently, and therapeutic experiments are now proceeding which will 
prove whether this one class of idiots may possibly be benefited by treat- 
ment. Until a year or two ago there was but little more hope of improving 
myxedematous patients than of helping other congenital idiots, and a removal 
from the mountainous region in which cretinism was endemic, or an en- 
tire change of surroundings, was supposed to be the only possible way of 
procuring some relief, though actual cure of any such condition had not, to 
my knowledge, been recorded by anyone. 

As will be seen in the discussion of the treatment of this condition there 
is some hope of improving these children by the use of recent methods. 

Treatment. — All the older suggestions with regard to treatment can 
be discarded. Feeding the patient with the thyroid gland promises some 
relief. The author has been personally interested in the treatment of four 
patients with myxedematous idiocy. In one of them a very decided change 
was effected, the mental and physical symptoms being greatly benefited by 
the use of thyroid extract. In two other cases the treatment was entirely 
unsuccessful, and in a fourth patient, a cretin, aged thirty-four years, a con- 
siderable change in the physical condition was effected, but the patient 
became so emaciated that the drug had to be discontinued.! The pulverized 
gland is now for sale in the market and can be obtained easily in any 
quantity. With children the treatment should be begun with doses of one 
grain of the thyroid gland, to be given three times a day, and increased from 
one up to five grains. While the drug is being given the child should be 
under proper medical observation, and the effect of the administration of 
the gland upon the heart and other organs should be carefully noted. This 
treatment should be begun in the earliest stages of myxedematous idiocy, 
with the view to prevent, if possible, 'the full development of the disease. 

* For the pathology of this condition, see page 206. 

t All these cases were treated more than a year ago. With the improved method 
of administering the " gland," better results may be expected. 



IDIOCY AND IMBECILITY. 647 



BIBLIOGRAPHY. 

Bourneville: Recherches, etc. Paris, 1890. 

Bourneville et Noir : Arch, de Neurol., Paris, 1893. 

Clouston : Neuroses of Development. Edinburgh, 1894. 

Down : Mental Affections, etc. London, 1887. 

Emminghaus : Die psychischen Storungen, etc., p. 243, Tubingen, 1887. 

(Literature of subject.) 
Ireland: Idiocy and Imbecility. London, 1887. 
Knight, G. H. : Johns Hopkins Bulletin. Baltimore, 1894. 
Krapelin : Arch. f. Psychiatrie, etc., Bd. XIII., p. 382. 
Meltzer : New Yorker Monatsschrift, April, 1894. (Myxcedema.) 
Mills : In Starr's Text-Book of Diseases of Children, p. 682. 
Northrup : Archives of Pediatrics, 1894. (Sporadic cretinism.) 
Otto: Arch. f. Psychiatrie, Bd. XXIII. , p. 153. 
Sachs: Hamilton's Legal Medicine, vol. ii., 1894; Journal of Nervous and 

Mental Diseases, 1887. 
Shuttleworth : British Medical Journal, January 30, 1886. 
Sinkler : Philadelphia Hospital Reports, 1893, ii., 161, 163. 
Wildermuth: Wiirtemberg Med. Corresp.-Blatt., 1886, No. 46, quoted by 

Emminghaus. 
Wilmarth : Alienist and Neurologist, 1890. 



APPENDIX. 

A FEW THERAPEUTIC SUGGESTIONS. 



APPENDIX. 

A FEW THERAPEUTIC SUGGESTIONS. 

The treatment of the various nervous disorders of childhood has been 
fully considered in the preceding chapters ; but several important therapeutic 
methods could not be given in detail in the body of this treatise. It is es- 
sential, however, that the physician be thoroughly familiar with them. To 
the account of these special methods the author will add brief remarks on the 
use and abuse of a few medicinal agents. 

The Rest Cure* was orginally devised by Weir Mitchell, for the treat- 
ment of neurasthenia and hysteria, chiefly in women. It is applicable to 
children, and the author has used it most freely for the cure of many chronic 
nervous disorders in the young, such as chorea, epilepsy, hysteria, hypochon- 
driasis, exhaustion following masturbation, and mild forms of melancholy. 

The chief features of this treatment are : Isolation, a good nurse, abso- 
lute rest in bed, nutritious diet, massage, electricity, hydrotherapeutic meas- 
ures. 

Isolation is the first essential of success, and often the most difficult to 
procure. It is best to remove the patient to a private sanitarium or a private 
hospital. In large hospitals it is more difficult to obtain sufficient attention 
to details, and the general commotion in hospitals is not conducive to the 
quiet of the patient. If parents object to the removal of the patient, isola- 
tion may be effected by putting the child in a top-floor room. No one but 
the nurse is allowed in the room ; meals are brought to the door. The first 
good effect of isolation is to impress upon the patient that he or she is to be 
subjected to strict discipline, and that the entire treatment is to be taken seri- 
ously. The selection of the nurse is an important matter. Relatively few 
nurses are fitted to take charge of these cases. It requires tact, patience. 
good judgment, and a scrupulous attention to the directions of the physician. 
The nurse who insists on what she calls " busy cases " is the last one to be 
chosen. She must also be in sympathy with a child's ways without being 
over-indulgent. I prefer to place male nurses over boys past the age of ten 
years. Some of the former are quite as competent as female nurses are. 

Children often object to this enforced seclusion for the first few days, but 

* For full details see S. Weir Mitchell in American Clinical Lectures, vol. i. : also 
Fat and Blood, fifth edition, 1888. J. K. Mitchell, Hare's System of Practical Thera- 
peutics, vol. i. , p. 227. 



652 APPENDIX. 

yield as soon as they discover that the nurse is kind to them. In some in- 
stances I have allowed the mother, provided she be a sensible one, to relieve 
the nurse for an hour each day. The visit of the mother is to be regarded 
as a reward for strict observance of the doctor's and nurse's orders. 

Rest. — This is the most important point. It implies absolute rest in bed. 
To this the child invariably objects at first, but, like the adult, it feels so 
thoroughly contented after a few days that it has little desire to get up. I 
allow the patient to be placed on a lounge for half an hour in the morning 
and evening, and find that this little change is conducive to better sleep. 
As soon as a decided improvement has set in the child is allowed to sit up 
in bed for ten or fifteen minutes, according to its condition ; this time is 
gradually lengthened until the child sits up all day, with the exception of a 
prolonged rest in the middle of the day, upon which it is well to insist even 
after the entire course of treatment is ended. By degrees the patient is urged 
to take short walks, and is led back gradually to a healthful mode of living. 

Diet. — Mitchell insisted in former years on an exclusive milk diet. In 
children this can be enforced quite readily, but it is better, on the whole, to 
give a mild general diet, and in addition from one to two quarts of milk in 
twenty-four hours. If simple skimmed milk cannot be taken, peptonized 
milk, kumyss, or matzoon may be substituted. The main point is to give that 
form of diet which is the most nutritious and most easily digested. 

Massage * is given to counteract the disadvantages of the rest treatment. 
What the patient needs is a careful kneading of the entire body, which can 
be given either by the nurse or by a professional rubber. Mitchell advises 
giving the massage at first for fifteen or twenty minutes, later on for an hour 
or an hour and a half in several sittings. Such details can be modified to 
suit the individual case. I have found it practicable to give it to children 
about an hour after a bath in the morning. 

Electricity is the least important feature of the rest-cure. It is intended 
to supply exercise for muscles that are not in daily use, and to this end a 
general faradization of all the muscles may be employed. This should be 
given by the physician or by a competent nurse. The faradic current has a 
slightly stimulating effect which acts favorably upon the general condition of 
the patient. " With these two means — electricity and the kneading of the 
muscles — we get excessive waste of tissue. We supply this again by exces- 
sive feeding, which is made possible by the improved assimilation resulting 
from the promotion of the digestion and of the circulation, brought about 
by the rubbing and electricity." (John K. Mitchell.) 

The part played by the electrical current is rather doubtful. In the 
author's experience it has been made to yield to 

Hydrotherapeutic Procedures. — In the morning, an hour or more after 
the first light breakfast, I order cold douches, followed immediately by coarse 
friction of the skin. At night I am in favor of giving a wet pack, which acts 
as a sedative, and promotes sleep. The child is stripped naked and wrapped 

* For the use of Swedish movements in addition, the reader is referred to special 
works. 



A FEW THERAPEUTIC SUGGESTIONS. 653 

in a sheet wrung out of water, at a temperature of about 90 . It is rubbed in 
lively fashion for a few minutes, and then put in a dry hot blanket in which 
it may be allowed to fall asleep. The less the patient is disturbed by these 
procedures the more quickly sleep will ensue. The method may be varied 
a little ; the principle to be remembered is that we must endeavor to bring 
about a dilatation of the blood-vessels in the skin, and to maintain such a 
dilatation for some period of time. This peripheral hyperemia is followed 
by a slower blood-current through the brain, and a temporary cerebral anae- 
mia — the conditions most favorable to sleep. 

In every rest-cure some drugs, such as iron, arsenic, and strychnia, may be 
given. Malt and cod-liver oil are at times indicated. Mild hypnotics will 
be needed in some cases, as insomnia is often a troublesome symptom. The 
duration of the treatment will vary between four and ten or twelve weeks, 
according to the condition of the patient. 

Physicians are often at a loss to know how the time is to be divided. 
The following schedule, as I have modified it from Mitchell, for the treat- 
ment of children, will give the necessary details : 

7 a. M. — Milk ; to be followed one-half hour later by the morning's toilet. 

8 a. m. — Milk, one boiled tgg, toast. 

9 a. m. — Cold douches followed by friction of skin. Rest for one hour. 
10 a. m. — Eight ounces peptonized milk. 

10.30 A. M. — Massage (one-half hour). Rest until 
1 p. m. — Dinner, followed by absolute rest of one hour. 
2.30 to 3.30 P. m. — Can be read to, or can be allowed to play. 
3.30 P. M.— Milk. 

4 P. M. — Electricity, or one-half hour's massage. 

5 to 6 p. M. — Can be amused by some games. 

6 P. m. — Supper, consisting of milk, tgg, and toast. 

7.30 P. M. — Wet pack, after which patient is to fall asleep. Milk is to be 
given during the night if patient wakes up. 

Hydrotherapy.* — Among the general therapeutic measures none has 
proved more valuable in the treatment of the nervous diseases than hydro- 
therapy. 

As far as the nervous diseases of children are concerned we use the water 
treatment either for its tonic or its sedative effect, the former in all conditions 
of nervous exhaustion, of general weakness, and in cases of anaemia; the 
latter in conditions associated with excitement or attended by pain, and in 
cases of sleeplessness. The effect of water upon the body is traceable, first . 
to mechanical impact ; second, to temperature effects ; and both these actions 
are secured through the influence exerted upon the vasomotor nerves in the 
skin and other peripheral organs. Respiration and cardiac action are acceler- 
ated, and blood-tension is increased. Dr. Baruch states, " a brief and intense 
application of cold is a stimulant, because it is at once followed by a corre- 

*A11 the necessary information can be obtained from Dr. S. Baruch's able article 
in Hare's System of Practical Therapeutics, and his monograph on The Uses of Water, 
etc., 1892. 



654 APPENDIX. 

sponding reaction, while a prolonged application is a depressant." Intense 
cold and excessive heat applied to the body have similar effects. All the 
effects of hydriatic treatment can be secured very much better at special 
institutions ; but these are very rare as yet, and many patients are exhausted 
by the effort following upon attendance at such establishments. We are 
compelled to resort, therefore, to such treatment as can be given at the pa- 
tient's home. 

Ablutions : A liberal application of water to the skin with the sponge or 
bare hand is particularly useful in febrile conditions. The temperature of the 
water is to vary gradually from 85 to 70 F. Fever is not only reduced by 
this method, but a stimulating effect upon respiration and circulation is 
noticeable. A similar effect may be gained by the sheet-bath. The patient 
is wrapped in a linen sheet, wrung not quite dry out of water at a temperature 
from 6o° to 70 F. The sheet is made to cling to the entire body by passing 
the flat hands over it. As the patient lies on this sheet water is poured over 
various parts of the body and rapid passes are made over these parts. The 
patient may be chilled but should not shiver. 

The wet pack is a damp sheet by means of which we endeavor to envelop 
the patient in a vapor-bath of his own creation. " If the pack is prolonged 
to several hours, so long as the sheet retains a temperature below that of the 
body the continuous flow of warm blood to the periphery excites cutaneous- 
nerve stimulation." . . . ." A sheet wrung from water at a temperature of 
6o D F. on a patient who has been previously warmed up (not by active exercise, 
however) will bring a reaction much sooner than one wrung out of water at 
8o° F." (Baruch.) The wet pack is particularly serviceable in ansemic children 
whose general condition is below par, and as a hypnotic measure. 

The dripping sheet may be used as a tonic measure in chronic nervous 
disorders (both functional and organic). The patient is asked to stand in a 
tub containing about twelve inches of water at 98 F. ; a coarse linen sheet is 
dipped in water at 70 F., which is reduced daily two degrees until 48 F. is 
reached ; with children we may not be able to go down below 6o° F. or 55 F. 
This sheet is thrown over the patient from behind, covering both head and 
body. Rapid passes with both hands are made, and the surface of the body 
is rubbed and slapped with outstretched hands. The effect of the drip-sheet 
is accounted for by the instantaneous stimulation of the sensory nerves of 
large areas of the body. Respiration is deepened, and the blood circulates 
more slowly through the lungs. 

Weir Mitchell* has given the following memoranda for the use of the 
drip sheet as a sedative at bedtime. "A basin of water at 65 F. Lower 
the temperature day by day by degrees to 55 F, or to still less. Put in the 
basin a sheet, letting the corners hang out to be taken hold of. The patient 
stands in one garment in comfortably hot water. Having ready a large soft 
towel and iced water, dip the towel in this, wring it and put it turban wise 
about the head and back of the neck. Standing in front of the patient, the 
basin and sheet behind, the maid seizes the wet sheet by the two corners 

* Medical Record, December 24, 1892. 






A FEW THERAPEUTIC SUGGESTIONS. 655 

and throws it around the patient, who holds it at the neck. A rough, smart, 
rapid rub from the outside applies the sheet everywhere. This takes but 
two minutes or less. Drop the sheet, allow the patient to lie down upon a 
lounge upon a blanket, wrap her in it, dry thoroughly and roughly with 
coarse towels placed at hand. Wrap in a dry blanket, remove ice- wrap, dry 
her, put on nightdress ; bed, the feet covered with a flannel wrap." 

This method may be tried with children near the age of puberty. Younger 
children would find it too severe. 

Full baths * at body temperature evidently have a soothing effect upon 
the entire nervous system including the brain. With a mild dilatation of the 
vessels in the skin a corresponding anaemia, or at least a slower blood-rate in 
the brain, is produced. These full baths are particularly useful in cases of 
cerebral excitement and insomnia. 

It remains for us to refer to a few drugs commonly used in nervous 
disorders : 

Arsenic : To be given in the form of Fowler's solution, four to ten 
drops in a large quantity of water ; excessive doses to be avoided. Older 
children may take arsenious acid (one-sixtieth to one-thirtieth grain) in pills. 

Aconitia (Dusquenef's) is used very little in early years ; to be given only 
in severe neuralgia and tachycardia. Dosage from one two-hundredth to 
one one-hundredth grain ; to be used very cautiously. 

Amy I nitris. (nitrite of amyl), with equal parts of chloroform, to be inhaled 
at the beginning of an epileptic seizure. (Four to six drops of the mixture.) 

Antipyrin, two to live grains ; antifebrin, one to three grains ; both drugs 
to be used very sparingly in children ; they are not needed. Phenacetin in 
two to five grain doses, with citrate of caffein (one-half to one grain), can be 
exhibited instead for the cure of headaches. Citrate of caffein alone is still 
better (one-half to one grain), every hour or two, until relieved. 

Atropines sulphas, one one-hundred and eightieth to one one-hundredth 
grain, in some cases of epilepsy, and in some spasmodic affections. 

The Bromides of Potassium, Sodium, and Ammonium. — The drugs most 
abused. These salts can be used singly or in combination, the total dose to 
vary between five to fifteen grains, three times daily. They are specially indi- 
cated in epilepsy and in some states of mental excitement. In most function- 
al nervous disorders (such as neurasthenia and hysteria) they are worse than 
useless ; as hypnotics they have very little effect. The custom of giving bro- 
mides whenever a person is " nervous " is to be condemned utterly. The mo- 
nobromide of camphor, two to five grains, is useful in some cases of epilepsy. 

Caffein: Valuable in headaches (see remarks on antipyrin), also as a 
heart-tonic; used in combination with cannabis indica (Hering's extract, gr. 
-fe to gr. £) in migraine. 

* Half-baths (tub containing about eight inches of water, in which the patient sits), 
the free use of douches, and friction to various parts of the body, arc more beneficial in 
the chronic disorders of the adult than in any of the chronic nervous diseases of child- 
hood. For special methods practised in hydriatic institutions the reader is referred 
to the works of Winternitz (Die Hydrotherapie, Vienna, 1890) and of Baruch. 



656 . APPENDIX. 

Ferrum : Iron should be given as ferrum redactum, or in Blaud's pills ; 
also in one of the many peptonates of iron now in the market. The combina- 
tion of iron and manganese is useful in girls at the beginning of menstruation. 

Hydrargyrum : In children mercurial treatment should be accomplished 
by means of inunction of unguentum hydrargyri (one-half to one drachm, 
daily) to be rubbed in thoroughly, very much as in adults. Oleatum hydrar- 
gyri may be substituted. Hypodermatic injections of sublimate are to be 
avoided. Mercurials are to be exhibited not only in specific disease (hereditary 
or acquired) but in many of the acute exudative diseases of the nervous system. 

Calomel is useful as a purgative, and particularly in the early stages of 
cerebral and spinal affections. 

Hyoscinum Hydrobro7iiatum (one two-hundredth to one one-hundredth 
grain). A most useful drug in all cases of cerebral excitement ; can be given 
by the mouth or hypodermatically ; to be used with great caution. 

The Iodides of Potash and of Sodium ; the latter to be preferred on ac- 
count of its indifferent action upon the heart ; both are to be given in satu- 
rated solution, four, ten, to fifty drops three times daily, according to the effect 
desired. The drops are given best in a large quantity of alkaline water or 
milk before meals. The dosage should be increased very slowly, and the 
condition of stomach and bowels closely observed. 

Morphium and all its salts are to be used as little as possible. There is 
in children, as well as in adults, a danger of the formation of the morphine 
habit. Very small doses (one-twentieth to one-tenth grain) to be exhibited 
according to the age of the child. No excuse for its persistent or occasional 
use except in severe neuralgias, in convulsions, and in some forms of mental 
disturbance. 

Phosphorus is administered best either in the form of Thomson's 
Solution (five to fifteen or twenty drops), or in drop doses (one to three) of 
oleum phosphoratum. 

Strychnia is one of the few indispensable drugs in neurological practice. 
We may give either the sulphate of strychnia (one-ninetieth to one-fiftieth 
grain) in pills or in solution ; or the tincture of nux vomica (one to three 
drops). It is an excellent tonic for the nervous system, and at the same 
time stimulates the heart and the gastro-intestinal mucous membrane. It can 
be given hypodermatically, but this method has been much abused, par- 
ticularly in cases of cerebral and spinal palsies, in which little can be expected 
of the drug. 

Sulfonal a7id Trz'onal are the most reliable hypnotics. The latter acts 
more promptly than the former. Both drugs should be given (to children) in 
five to ten grain doses an hour before bedtime, in soup or warm milk. A 
natural sleep is apt to follow, with only very slight discomfort the next morn- 
ing. There is little danger of forming a habit. Chloralamid is the only 
other hypnotic which compares favorably with these. Urethan, in doses of 
about three to five grains, has been given successfully to young infants. The 
author has used sulfonal, trional, and chloralamid in nocturnal epilepsy 
whenever he wished to avoid an excess of bromides. 



INDEX. 



INDEX. 



Abducens nerve, in tumor of cerebel- 
lum, 566 
Ablutions, 653 
Abscess of brain, 576 

of cerebellum, 578 
Aconitia, 654 

Acute ascending paralysis. See Lan- 
dry's paralysis. 
Adiaemorrhysis, 470 
Agenesis corticalis, 399, 601 
Agoraphobia, 613 
Agraphia, 478 
Alexia, 478 
Amyelia, 439 
Amyl nitrite, 655 

Amyotrophic lateral sclerosis with bul- 
bar symptoms, 512 
Amyotrophies and myopathies, distinc- 
tion between, 405, 406 
Anaemia, headaches due to, 171 

of brain, 470 

of spinal cord, 284 
Anaesthesia, 37 

hysterical, 98 
Aneurism, cerebral, 572 
Angio-neurotic oedema, 207 
Angio-paralytic, angio-spastic forms of 

migraine, 183 
Ankle clonus, 40 
Anosmia (loss of smell) in tumor of 

frontal lobe, 561 
Antipyrin, 655 
Antitoxin of tetanus, 156 
Aphasia, 471 

in migraine, 181 

motor and sensory, 473 

occurrence of, 480 
Aphonia, hysterical, 94 
Apraxia, 480 

Aran-Duchenne type of progressive mus- 
cular atrophy, 407 
Arm, palsies of, 221 
Arsenic, 654 

method of administering in chorea, 127 
Arteries, of the brain, 464 

of the spinal cord, 2S1 
Astasia-abasia, 93 
Atelomyelia, 439 



Athetosis, 535, 538. See also "Double 

athetosis." 
Atrophy. See Progressive muscular 

atrophies. 
Atropine, 655 

Auditory nerve. See Hearing. 
Auditory symptoms in epilepsy, 67 
Aura, in epilepsy, 67 

Basal Ganglia, tumor of, 563 
Basedow's disease, 197 

cardinal symptoms of, 198 

theories of, 202 

treatment of, 203 
Baths, efficacy of, in chorea, 126. See 

also Hydrotherapy. 
Bell's paralysis, 229 
Birth palsies, 529 

morbid anatomy of, 540 
Blepharospasm, 238 
Blood-supply of brain, 464 

of spinal cord, 281 
Blood-vessels, dilatation of, in the brain 

in chorea, 121 
Bouche de tapir in progressive muscular 

atrophy, 427 
Brain abscess, 576 

symptomatology of, 579 
Brain, anatomy, physiology, and pathol- 
ogy of, 445 

blood-supply of the, 464 

defective development of, 589 

development of, 1 

diseases of, 445 

embolism of, 470 

hemorrhage in, 470 

hyperemia of, 470 

hypertrophy of, 602 

localization, 445 

of tumors in the, 470 

thrombosis in the, 470 

weight of, 471 
Bromides, ('55 

mode of administering in epilepsy, ~o 
Brown-Sequard's paralysis, with tumors 
oi spinal cord, 366 

in syphilis of the spinal cord. 334 
Bulbar palsies. 5 to 



66o 



INDEX. 



Caffein, 655 

Calomel, 655 
Caput obstipum, 15 
Catalepsy, 622 
Centres in the brain, 449 
Cephalic tetanus, 151 
Cerebellar type of hereditary ataxy, 390 
Cerebellum, tumor of, 566 
Cerebral development, arrest of, 396 
Cerebral diplegia, family forms of, 398 
Cerebral neurasthenia, 613 
Cerebral palsies, 523 
Cerebral tumors, 556 
Cerebral type of hereditary spastic par- 
alysis, 396 
Cerebro-spinal meningitis, 496 
Cervical segments in plexus lesions, 222 
Charcot - Marie type of progressive 

muscular atrophy, 411 
Chorea, complications of, 117 

duration of, 119 

electrical reactions in, 116 

germ theory of, 124 

hereditary form of, 131 

hygienic care in, 129 

mental disturbance in, 116 

morbid anatomy and pathology of , 120 

relapses in, 11S 

treatment of, 125 
Chorea electrica, 138 
Chorea insaniens, 116 

of Sydenham, 109 
Choreiform diseases, 131 
Choreiform movements, after cerebral 

palsies, 535 
Circular insanity, 621 
Clarke's column, 26S 
Clonus, ankle, 40 
Color-sense, in hysteria, 100 
Column of Gowers, 272 
Columns of Burdach and of Goll, 272 
Complex tics, 136 
Compression of spinal cord, 357 
Congenital diplegias, 399 
Congenital paramyotonia, 143 
Conjugated paralysis, 12 
Contracture in cerebral palsies, 533 
Contracture in myelitis, 316 
Contracture rheumatismal des nourrices, 

160 
Convulsions, 49 

causes of, 50 

diagnosis of, 60 

frequency of, 54 

in epilepsy, 66 

in hysteria, 88 

in lead-poisoning, 260 

partial, significance of, 56 

prognosis, 61 

theories of, 53 

treatment of, 62 
Convulsive tremor, 144 



Coprolalia, 139 

Cornu ammonis, disease of, as a cause 
of epilepsy, 75 

Corpora quadrigemina, tumor of, 563 

Cortex cerebri, 450 

Cortical tumors, 560 

Cranial measurements, 5 

Cranial nerve nuclei, defective develop- 
ment of, 603 

Craniotabes, 58 

Craniotomy, in infantile cerebral palsies, 
554 
in idiocy, 644 

Cretinism, sporadic, 644 

Crus cerebri, tumor of, 563 

Cyclops, 589 

Cysts of brain, in infantile cerebral 
palsy, 546 

Defective development of brain, 589 
Deformities in infantile spinal paralysis, 

296 
Degeneration, in the brain, 463 

in idiocy, 63S 

in insanity, 629 

reaction of, 44 
Dementia, 623 

in hereditary chorea, 132 
Diet, in chorea, 126 

in rest cure, 652 
Dietary, in epilepsy, 81 
Diphtheritic paralysis, 253 

course and duration of, 255 

pathological anatomy of, 256 
Diplegia, 523 
Diplomyelia, 440 
Diplopia, 12 
Disseminated sclerosis, 345 

atypical forms of, 351 

differentiation from paralysis agitans, 

353 

etiology of, 34S 

pathology of, 349 

prognosis, 354 

symptoms of, 345 

treatment of, 354 
Dolichocephalus, 6 
Double athetosis, 538 
Duchenne's type, 407 
Dystrophies, progressive muscular, 421 

Ear Disease, as a cause of headache, 
175 ; of abscess, 577 ; of sinus 
thrombosis, 586 
Echolalia, 139 
Eclampsia infantum, 49 
Electrical conditions, table of, 45 
examination, 42 

examination in progressive neural mus- 
cular atrophy, 414 
reactions in chorea, 116 



INDEX. 



66 1 



Electrical reactions in progressive mus- 
cular atrophies, 429 
Electricity in rest cure, 652 
Electricity, service of, 45 

use of, in chorea, 128 
Embolism in chorea, 122 
Encephalitis, acute, 508 
Enuresis nocturna, 195 
Epidemic cerebro-spinal meningitis, 496 

symptoms of, 499 
Epilepsia nutans, 137 
Epilepsy, 65 

aune in, 67 

causes of, 69 

diagnosis of, 72 

Jacksonian, 67. See Partial Convul- 
sions. 

pathological anatomy of, 75 

pathology of, 77 

prognosis of, 74 

psychic symptoms of, 69 

surgical treatment of, 81 

symptoms of, 65 

temperature in, 68 

treatment of, 78 ; Flechsig's method, 
80 

varieties of, 66 
Epileptic aura, treatment of, 81 
Epileptic insanity, 626 
Epileptiform convulsions, diagnosis from 

chorea, 120 
Erb's palsy, 223 

Erb's type of progressive muscular atro- 
phy, 426 
Essential paralysis of children, 289 
Eudiaemorrhysis, 470 
Examination, method of, 2 

scheme of, 3 
Exophthalmic goitre, 197 
Eye-strain, cause of headache, 178 ; re- 
lation to migraine, 185 
Eyes, conjugate deviation of, 69 

muscles of, no 

Face, asymmetry of, 12 

motor points of, 17 
Facial hemiatrophy, 210 
Facial palsy, 229 

a form of obstetrical palsy, 228 

electrical changes in, 231, 232 

treatment of, 236 
Facies myopathique in progressive mus- 
cular atrophy, 427 
Facio-scapulo-humeral type of progres- 
sive muscular atrophy, 426 
Ferrum, 655 
Fissures, cerebral, 446 
Flechsig's treatment of epilepsy, So 
Friedreich's disease, 37S 

diagnosis of, from hereditary chorea, 

133 
differential diagnosis of, 3S5 



Friedreich's disease, pathological anat- 
omy of, 386 
reflexes in, 379 
symptoms of, 379 

Gait, examination of, 35 

Gangrene, symmetrical, 207 

Gastric disturbances, headache due to, 

175 
Genital irritation, headaches due to, 175 
Glioma of brain, 511 
Gliosis of spinal cord, 373 
Glosso-labio-pharyngeal paralysis, 512 
Graefe's symptom, 200 
Grand mal, 66 
Graves's disease, 197 
Gyrospasms, 137 
causes of, 137 



Habit chorea, 136 
Headaches, 170 

a symptom of brain tumor, 558 

classification of, 170 

in organic disease of the brain, 176 
Head pains, location of, 171 
Hearing, examination of, 12 

centre of, 462 
Heart, affections of, in chorea, 117 
Hemianesthesia, 40 

hysterical, 98 
Hemianopsia, 9 

explanation of, 461 

in cerebral palsies, 533 
Hemiatrophy of face, 210 
Hemichorea, 114, 118 
Hemicrania, 179 

Hemiplegia, 523, 526, 529, 534, 542 
Hemorrhage, cerebral, 470, 542 
Hereditary and family affections, prelim- 
inary classification of, 401 
Hereditary ataxy, 37S, 390 
Hereditary chorea, 131 

differential diagnosis from post hemi- 
plegic chorea, 133 

pathological anatomy of, 134 

symptoms of, 132 
Hereditary chorea, without dementia, 

135 
Hereditary diseases of spinal cord, 377 
Hereditary spastic paralysis, 391 
the cerebral type of, 396, 530 
spastic paralysis of spinal origin, 305 
Heredity, influence of, in insanity, 609 
Heredo-ataxie cerebelleuse, 300 
Heterotopia, 430 
Huntington's chorea, 131 ; prognosis in, 

135 

treatment of, 135, 
Hydrargyrum, 055 
1 [ydrocephalus, 514 

a cause of epilepsy, 75 



662 



INDEX. 



Hydrocephalus, acquired internal, 519 

chronic, 516 

congenital, 517 

indications of, 6 

operative procedures for, 522 
Hydrorrachis externa, 440 
Hydro-therapeutic procedures, in rest 

cure, 652 
Hydrotherapy, 653 

Hygiene, importance of, in chorea, 129 
Hyoscinum hydrobromatum, 655 
Hypaesthesia, 37 
Hyperemia, headaches due to, 174 

of brain, 470 

of spinal cord, 2S6 
Hyperesthesia, 40 
Hyperdiremorrhysis, 470 
Hypochondriasis, 615 
Hypoglossal nerve, spasm of, 239 
Hysteria, 85 

diagnosis of, 102 

duration of, 103 

irregular manifestations of, 92 

manifestations of, 86 

motor manifestations of, SS 

muscular paralysis in, 93 

pathology of, 103 
Hysteria, psychic or mental, S6 

treatment of, 104 

visceral symptoms of, 100 
Hysterical anaesthesia, transferrence of, 

99 
anorexia, 100 
chorea, differentiation from hereditary 

chorea, 134 
insanity, 626 
paralysis, differentiation from acute J 

myelitis, 324 
Hystero-epilepsy, diagnosis of, 91 
relationship to true epilepsy, 88 
Hysterogenic zones, 9S 

Idiocy, 631 

acquired, 633 

classification of, 632 

developmental, 633 

hereditary congenital, 632 

signs of degeneration in, 638 

with epilepsy, 537 
Imbecility, 631 

Imperative conceptions, 139, 611 
Imperative movements, 137 
Infantile cerebral palsies, 523 

classification of, 540 

differential diagnosis, 54S 

distribution of, 526 

due to embolism and thrombosis, 545 

due to hemorrhage, 542 

etiology of, 528 

form of palsy, 531 

morbid anatomy, 539 

prognosis of, 549 



Infantile cerebral palsies, treatment, 550 

with epilepsy, 536 

with idiocy, 537 
Infantile oculo-facial palsy, 604 
Infantile spinal paralysis, 2S9 

morbid anatomy and pathology of, 298 

prognosis in, 307 

symptoms of, 2S9 

theory of, 303 

treatment of, 30S 
Infectious disease, the cause of menin- 
gitis, 506 

headache due to, 177 
Inflammation of nerves, 217, 240 
Influenza, the cause of meningitis, 507 ; 

of encephalitis, 508 
Insanity, 60S 

etiology of, 609 

forms of, 611 

frequency of, 609 

treatment of, 629 
Insomnia, 191 ; in polyneuritis, 252 
Insufficiencies, ocular, relation to head- 
ache, migraine, 17S, 185 
Intra-cranial sinuses, thrombosis of, 5S5 
Iodides, 655 

Jackson iax (partial) epilepsy, 67 
Jaw-jerk, 40 
Jumpers, 140 

Juvenile form of progressive muscular 
atrophy, 426 

Knee-jerk, 40 

Landouzy-Dejerixe type of progres- 
sive muscular atrophy, 426 

Landry's paralysis, 332 

differentiation from multiple neuritis, 

250 
differentiation from acute myelitis, 323 

Lannelongue's operation, 597 

Lanterman, incisures of, 217 

Laryngeal chorea, 115 

Laryngismus stridulus, 57 

Lead encephalopathy, 260 

Lead paralysis, 259 

Leg, palsies of, 238. See also Para- 
plegia. 

Leg type of progressive muscular atro- 
phy, 411 

Lenticular nucleus, affection of, in 
chorea, 123 

Leptomeningitis. See Meningitis. 

Leptomeningitis, specific, 333 

Lid reflex, light reflex, 10 

Lissauer's tract, 271 

Little's disease, diagnosis of, from hered- 
itary spastic paralysis, 396 

Localization in cortex, 445 
in spinal cord, 277 
of tumors, 560 



INDEX. 



66 3 



Macewen's symptom, 560 
Macrocephalus, 602 
Main en griff e, 27 
Maladie des tics convulsifs, 139 
Malaria, headaches due to, 177 
Malformations and defective develop- 
ment of the cord, 439 ; of the brain, 

589 
Mania, 616 

Massage in rest cure, 652 
Masturbation and insanity, 628 
Megrim, 179. See Migraine. 
Melancholia, 615 

attonita, 620 
Membranes, of the brain, blood-supply 
of, 464 

diseases of. See Meningitis and other 
diseases of brain. 
Membranes of the spinal cord, blood- 
supply of, 281 

tumors of, 364 
Meningeal hemorrhage at birth, 541 
Meninges. See Membranes. 
Meningitis, 4S3 

acute, 483 

epidemic, 496 

forms of, 483 

and influenza, 507 

and otitis media, 504 

serosa, 515 

treatment of, 487, 495, 502 

tubercular, 489 
Meningocele, 441 
Meningo-myelitis, specific, 333 
Meningo-myelocele, 441 
Mental disturbance in chorea, 116 
Mental excitement, control of, in chorea, 

128 
Meynert's commissure, 460 
Microcephalus, 594 
Migraine, 179 

diagnosis of, 1S6 

etiology of, 182 

ocular insufficiencies in, 184 

pathology of, 183 

periodicity of, 183 

prognosis of, 186 

relation of, to epilepsy, 184 

treatment of, 187 
Monoplegia, cerebral, rarity of, 320 
Moral insanity, 625 
Morphium, 655 
Motor area, anatomy of, 449 

tumor of, 561 
Motor cortex, condition of, in spastic 

paraplegia, 400 
Motor points, 17, 23, 25, 29, 32, 33 

tract, 450 
Multiple neuritis, 240 

forms of, 246 

pathological anatomy of, 248 

symptoms of, 240 



Multiple neuritis, treatment of, 251 
Multiple sclerosis, diagnosis from Fried- 
reich's disease, 386. See also dis- 
seminated sclerosis. 
Muscles of arm, forearm, and hand, 22 

of back and trunk, 33 

of the head and neck, 1 5 
- of the eyes and face, 10, 11 

of pelvic girdle and lower extremities, 
28, 30 

of shoulders and upper extremity, 16 

of the tongue, palate, and pharynx, 13 

total absence and early atrophy of, 436 
Muscular hypertrophy, physiological, 429 

irritability in Thomsen's disease, 142 

movements in hereditary chorea, 132 
Muscular pseudo-hypertrophy, 423 
Muscular sense, 37 
Muscular strength, tests of, 34 
Mutism, hysterical, 94 
Mydriasis, 1 1 
Myelitis, acute, 313 

differential diagnosis, 322 

pathology and morbid anatomy of, 319 

specific, 333 

symptoms of acute form, 313 

treatment of, 325 
Myosis, causes of, 12 
Myotonia congenita, 141 
Myotonic reaction, 141 
Mysophobia, 612 
Myxcedema, 205 

treatment of, 207 
Myxedematous idiocy, 206, 644 

Nerves, cranial, nuclei of, 462 

degeneration and inflammation of, 
218 

facial, disease of, 229 

motor, of the eye, 10 

of arm, forearm, and hand, 22 

of back and trunk, 33 

of head and neck, 15 

of pelvic girdle and lower extremities. 
36 

of shoulders and lower extremities, 16 

of tongue, palate, and pharynx, 14 
Nerve-trunk, anatomy of, 217 
Nerve-trunks, inflammation of, 219, 220, 

221 
Nervous system, organic diseases of, 215, 
216 ; general functional diseases, 40 
Neurasthenia, cerebral type of, 613 

headache due to, 173 
Neuritis, 217, 240 

Neuroglia, hyperplasia of. in epilepsy, 7c 
Neuron, 26S 
Nictitation, 238 
Nodes of Ranvier, 217 
Nonne-Marie type of cerebellar ataxy, 

300 
Nystagmus, o 



664 



IXDEX. 



Obstetrical palsies, 223 

diagnosis of, 224 
pathology of, 224 
treatment of, 227 
Occipital lobe, defective development of, 

599 
tumor of, 561 

CEdema, angio-neurotic, 207 

Olfactory tract, 461 

Ophthalmoplegia, externa and interna, 
12 
partial and complete, 510 

Opisthotonus, 147, 162 

Optic thalamus, affection of, in chorea, 
123 

Otitic disease, relation of, to brain ab- 
scess, 577. See Ear disease. 

Oxycephalus, 6 

Pain, 36 

Palsy. See Paralysis. 
Paradoxical contraction, 40 
Paraesthesiae, 220 

Paralysis, distribution of, in infantile 
cerebral palsies, 526 

Erb's, 223 

facial, 229 

infantile spinal, 289 

in peripheral nerve disease, 217 

in spinal palsies, 2S9 

of ocular muscles in disseminated scle- 
rosis, 348 
Paramyoclonus multiplex, 143 
Paranoia, 623 
Paraphasia, 478 
Paraplegia, spastic cerebral, 523 

spastic spinal, 393 
Paretic dementia, 627 
Parietal lobe, tumor of, 562 
Patellar reflex, 40 
Pavor nocturnus, 193 
Periodic insanity, 621 
Peripheral nerves, diseases of, 217 

palsies, 217 
Peroneal paralysis, 233 

form of progressive muscular atrophy. 

Phosphorus, 655 
Pleuroplegia, 605 

congenital, 604 
Polio-encephalitis superior, 509 
Polio-encephalomy elitis ,510 
Poliomyelitis anterior acuta, 289 
Polyneuritis, 240. See Multiple neu- 
ritis. 
Pons, tumor of, 565 
Porencephaly, 590 

acquired, 593 

congenital, 591 
Post-hemiplegic chorea, 119, 535 
Pott's paralysis, 3, 357 

diagnosis of, 360 



Pott's paralysis, symptoms of, 359 
Primary dystrophies, types of, 421 

muscular dystrophies, 420 

myopathies, 420 
Progressive amyotrophy, 407 
Progressive muscular atrophies, duration 
of, 435 

pathology of, 431 

hereditary form, 409 

muscular atrophy, 404, 407 

muscular atrophy, abortive and hered- 
itary forms of, 411 
Progressive neural muscular atrophy, 411 

neurotic muscular atrophy, 411 

pathology of, 41 3 
Pseudo-hypertrophic muscular paralysis, 

424 ' 
Ptosis, 11 



Raxvier, nodes of, 217 
Raynaud's disease, 209 
Reaction of degeneration, 44 
Reflex of cornea, test for, 10 
Reflexes, examination of, 40 

theory of, 2S0 

variations of, 41 
Regeneration of nerves, 219 
Rest, efficacy of, in chorea, 126 
Rest cure, 651 

schedule for, 653 
Rheumatic nodules in chorea, 117 
Risus sardonicus, 149 
I 
Sarcoma of brain, 571 
Scaphocephalus, 6 
Schwann, sheath of, 217 
Sclerosis, amyotrophic lateral, 512 
Sensation, disturbances of, in mi- 
graine, iSo 

tests for, 37 

varieties of, 37 
Sensory r disturbances, determination of, 

36, 37 
Sensory nerves,- distribution of, 38, 39 
Sensory tract, 270, 459 
Skull, abnormalities of, 6 

measurements of, 4 
Sleep, disorders of , 191 

normal amount of, 192 
Somnambulism, 196 
Spasms, 137 et seq. 
Spastic contracture, causation of, 280 
Spastic hemiplegia, 523 

diplegia, 396, 523 

paraplegia, 394, 523 

spinal paralysis, 394 
Speech defects, 471 

method of testing for, 451 
Speech, difficulties of, in maladie des tics 

convulsifs, 140 
Speech, disturbance of, in hereditary 
chorea, 133 



INDEX. 



66 s 



Speech, disturbances of, in chorea, 

US 
Spina bifida, 440 
Spinal cord, anaemia of, 284 

anatomy, physiology, and pathology 

of, 263 
asymmetry of, 434 
blood-supply of, 282 
changes of, in pernicious anaemia, 

285 
gliosis of, 373 
hereditary or family diseases of, 

377 

hyperemia of, 286 

localization in, 277 

malformations and conditions due to 
defective development of, 439 

structure of, 263 

syphilis of, 333 

traumatic injuries of, 328 

tumors of, 364 
Spotted fever, 496 
Stelhvag's symptom in exophthalmic 

goitre, 200 
Strychnia, 656 

Subacute anterior poliomyelitis, 310 
Subjective sensations, 36 
Sulfonal, 656 
Sydenham's disease, 109 
Syphilis of spinal cord, morbid anatomy 
of, 333 

symptoms of, 333 

treatment of, 342 
Syringomyelia, 373 
Syringo-myelocele, 441 

Tabes dorsalis spasmodique, 396 

Tachycardia, 205 

Teeth, significance of condition of, 13 

Temperature-sense, 37 

Temporo - sphenoidal lobe, tumor of, 

562 
Tendon reflexes, 40 
Tetanilla, 160. See Tetany. 
Tetanoid chorea, 168 
Tetanus, 147 

bacillus of, 147, 148, 154 

differential diagnosis of, 156 

etiology of, 147 

pathology of, 154 

prognosis of, 157 

symptoms of, 149 

treatment of, 157 

varieties of, 151 
Tetanus neonatorum, 147, 151 
Tetany, 160 

etiology of, 163 

latent period of, 164 

pathology of, 165 

symptoms of, 161 

treatment of, 1C7 
Thomsen's disease, 141 



Thrombosis, of intra-cranial sinuses, 

585 

of lateral sinus, differential diagnosis 
from brain abscess, 582 
Thyroid enlargement at puberty, 205 
Thyroid gland, extirpation of, in relation 
to tetany, 166 

feeding in myxcedema, 207, 646 
Tic convulsif, 140 
Tics, 136 
Topoalgia, 36 
Torticollis, spasmodic, 238 
Toxic headaches, 178 
Tracts, in the brain, 450 et seq. 

in the spinal cord, 270 
Traumatic injuries of spinal cord, 328 
Tremor, fibrillary, 406 

of multiple sclerosis, 345 
Trigeminal neuralgia, rarity of, 186 
Trional, 656 
Trismus, 147 

Trophic disturbances in peripheral neu- 
ritis, 221 
Trophic symptoms, occurrence of, 45 
Tropho-neuroses, 197 
Trousseau's symptom, 164 
Tubercular meningitis, 489 

morbid anatomy, 492 

onset, 489 

symptoms, 490 
Tumor of cortex, 560 

of basal ganglia, 563 

of cerebellum, 566 

of corpora quadrigemina, 563 

of crus cerebri, 563 

of medulla and pons, 565 

of motor area, 561 

of occipital lobe, 562 

of parietal lobe, 562 

of temporo-sphenoidal lobe, 562 

of third frontal convolution, 561 
Tumors of the brain and meninges, 

. 556 . 

differential diagnosis, 56S 
pathology of, 569 
treatment of, 572 
Tumors of spinal cord and meninges, 364 
diagnosis of, 369 
prognosis, 371 
symptoms of, 364 
treatment of, 371 

URAEMIA, headaches due to, 177 
Urine, changes in, in chorea, 117 

Vasomotor neuroses, 197 
Venous circulation in brain, 468 
Ventricle, fourth, stimulation of floor of. 

77 
Vertigo in migraine, 1S1 
Vision, disturbances of, in hysteria. 

100 



666 



INDEX. 



Vision, test for, in infants, 8 
Visual field, 9 



Water treatment. 

py- 

"W eight of brain, 471 
Weight-sense, 37 



See Hydrothera- 



Weir-Mitchell plan of treatment, 105 

Wet pack, 654 

Word-blindness, word-deafness, 474 

Wrist-drop, 21, 242 

Wry-neck, 238 

congenital, 238 

symptomatic, 238 



H" 11 88 






-^V v^V °v*^v v^> 











/ ♦* ^ 



<* *<T 






*0 






,♦ ... 




.^'. % 



«„ A" *'sh%?/K°~ *<. **'» v f3K'. +*« A*' ,V<yV* . '*,. A «'.1 






**<*' . 









\<* ; J%- \/ .*^\ v** .-ate-- \s : 


















°q,;^^f^ o 










1 *!,\?* > V % *I 






^■i- 






* V ""<► 



^>^T^ ,6* 



o. 14 















V ^^ .1 






* *^r • - y % 








'X ^ V> %ii^ 











y .^^ ^ 







4 °* 




<u 





















r.«* ,o 



G°*,C^**°o 




,4°*. 




... * A 



lP-A 







5°* 






-jxj- 











^0^ 




HECKMAN 

BINDERY INC. 



^ SEPT 88 

$=W' N. MANCHESTER, 
— INDIANA 46962 



NT^ 



